Shenlein-Genoch disease: pathogenesis, symptoms, recognition. Systemic vasculitis

Schonlein-Henoch disease in children and adults is a pathology that is accompanied by damage to the vascular walls. This is one of the varieties of vasculitis, which is the most popular and occurs more often in childhood.

It is expressed by inflammatory processes that occur in the vessels of the skin and internal organs. The disease is provoked by an activated component located in human body.

You can get this disease regardless of age, but in children under 3 years old it develops extremely rarely.

According to statistics, the disease makes itself felt, starting from 4 years and up to 12 years of age.

The initial stage of the disease, as a rule, falls on the first 3-4 weeks after an infectious disease, viral pathologies, etc. (tonsillitis, scarlet fever). Trigger for the appearance of the disease is a trauma, drug intolerance, an allergic reaction, and so on.

Schonlein-Henoch disease

The pathology is autoimmune. At the beginning of its development, many immune reactions accompanied by damage to the walls of blood vessels.

Why does

The culprit for the development of the disease is the transferred infection, as well as vaccines and various sera, which give an inadequate reaction.

In this case, the appearance of circulating immune complexes occurs, settling in the vessels and provoking their damage. As mentioned above, various bacterial or viral infections, and helminthic infestations, adverse environmental conditions.

Varieties

If we talk about the clinical picture, then it suggests division of the disease into acute and chronic course. According to the symptoms, Henoch-Schonlein disease is classified into:

• simple;
• articular;
• with abdominal syndrome;
• lightning-fast form.

According to the severity stage, there are:

• a mild degree, in which the patient's state of health is satisfactory and there are not pronounced rashes;
• moderate severity is manifested by a profuse rash, pathological changes in the joints, moderate abdominal pain;
• severe form of the disease is accompanied by severe clinical course, extensive lesions with necrotic areas, kidney damage, neurotic edema, as in the photo, in patients with abdominal syndrome, bleeding in the gastrointestinal tract is manifested.

Photo Schonlein-Henoch disease

If we talk about the acute form of the disease, then it cannot last longer than two months, the protracted stage lasts up to 6 months and the chronic form lasts longer than six months.

Clinical manifestations of the disease

Patients with hemorrhagic vasculitis are characterized by intense fever. But the increase in temperature is not always observed.

At the beginning of the development of the disease, the so-called skin syndrome makes itself felt. It is typical for absolutely all patients. Spotty and papular formations of various sizes appear on the skin, which disappear if you press on them. The rash is often symmetrical and manifests itself in the thighs, buttocks of the lower extremities. Rarely, in severe cases, tissue necrosis occurs.

Also, in some patients articular syndrome.Symptoms may be short-lived or last for days and be accompanied by intense pain, which can lead to limited movement of the joints.

Usually the lesion extends to large joints, in some cases the knees and ankles may be affected. Such a syndrome can develop at the initial stage of vasculitis. As a rule, its character is unpredictable, but it does not lead to deformation.

All of the above symptoms are accompanied by abdominal syndrome. Patients complain of severe pain in the abdomen. It is difficult for patients to answer the question exactly about the localization of pain, they also notice a violation of defecation, they feel sick and vomit.

The pain syndrome occurs spontaneously and stops in the same way. In advanced cases, intestinal obstruction develops.

Patients with pathology complain of severe pain in the abdomen

This disease leads to another serious lesion - nephrotic syndrome, which is the most persistent and is fraught with the appearance of kidney failure and can lead to such a disease as chronic nephritis.

As for other organs, they are affected very rarely. Myocarditis, pericarditis, pneumonia, etc. occur.

Damage to the brain and its membranes makes itself felt by confusion, apathy, fatigue, nervousness, headaches. The symptom is very serious and can lead to the development of meningitis.

How to make a diagnosis

First of all, you need to get an appointment with a doctor. The specialist pays attention to age, general condition, weighs the results of studies and the patient's stories about the symptoms, excludes other diseases.

A blood test usually shows non-specific manifestations of aseptic inflammation, eosinophils and platelets will certainly be increased. It is also necessary to donate urine and blood for biochemistry.

Be sure to do a coagulogram if you suspect Shenlein-Henoch disease.

In case of kidney damage, the patient should definitely consult a nephrologist doctor. The specialist will tell you which tests you need to pass.

Methods of treatment

The disease is treated with a whole range of measures. official medicine suggests starting treatment with a change in eating habits. All allergenic products are completely excluded. After that, the patient needs to be in bed. pharmaceutical treatment involves taking medications that prevent blood from clotting.

Therapy of organs and systems is required according to clinical signs in each individual order.

As a rule, anti-inflammatory drugs, hormonal agents and medicines are prescribed that strengthen the immune system.

It is important that children be registered. During treatment, they should not be in the sun, engage in active sports and undergo physiotherapy procedures.

Prevention measures

With timely and effective therapy the disease is successfully treated

To prevent the disease, it is important to recognize it in time and carry out effective treatment. Hardening and strengthening of immunity is important. You also need to regularly sanitize your home, install air purifiers and humidifiers, and ventilate the room to get rid of accumulations of bacteria and dirt. Children need to be tempered from childhood. The intake of any medication should be discussed with a specialist.

A patient with such a diagnosis should have proper nutrition and established day mode. If you follow the principles of a healthy lifestyle, then you will protect yourself not only from vasculitis, but also from many serious pathologies.

Forecast

Mild forms of the disease are successfully treated. However, the lightning current can end lethal outcome in the first days after the development of the disease, which is explained by vascular damage and the appearance of hemorrhage in the brain. Severe renal syndrome may also be among the causes of death.

Conclusion

Hemorrhagic vasculitis is a serious disease that requires an immediate response from the patient. It is important to seek medical help on time, otherwise unexpected consequences may occur. With the right actions, the disease is successfully treated and recedes, after which the person recovers and returns to a full life.

Schonlein-Henoch purpura (hemorrhagic vasculitis, Schonlein-Henoch disease, anaphylactoid purpura, hemorrhagic capillary toxicosis) is a systemic vasculitis affecting the vessels of the microcirculatory bed (arterioles, capillaries and post-capillary venules), with a characteristic deposition in their wall of immune deposits, consisting mainly of immunoglobulins A (IgA); clinically manifested by skin hemorrhagic rash in combination with damage to the joints, gastrointestinal intestinal tract and kidneys

William Heberden was the first to describe the disease in his Commentarii de Marlbaun, published in 1801. And in section 78 "Purpureae Maculae" of his other book "Commentaries on the History and Cure of Diseases", printed in 1802, he describes two clinical observations. subsequently yellow edema of the extremities, buttocks and scrotum. A 5-year-old boy with similar swelling and discoloration, especially of the legs. In addition, swelling and pain were noted in certain parts of the body, in particular, the penis was so swollen that urination was difficult. Periodically there were pains in the abdomen with vomiting, at the same time streaks of blood were found in the stool, and the urine was slightly stained with blood. With the appearance of pain in the legs, the boy could not walk, at the time of the examination, all legs were covered with blood-colored spots

Clement Ollivier In both cases, the disease resolved itself, although the duration was longer in the second case. In 1808, Robert Willan, who introduced the term "purpura haemorrhagica", described a very similar case in 1827, drew attention to the combination of purpura and abdominal pain, but this condition was not considered as an independent nosological form.

Johann Lukas Shönlein in 1837 called the combination of arthralgias and arthritis with macular rash "peliosis rheumatica" (livedo rheumatica). The exanthema in livedo rheumatica described by Shenlein did not cause hemorrhages in the skin or mucous membranes, but the disease could damage the internal organs and acquire a chronic course.

Eduard Heinrich Henoch In 1874 reported four children with bloody diarrhoea, abdominal pain and a rash associated with painful joints Both Schonlein and Henoch described the rash as macular rather than purpuric. Henoch also drew attention to the fact that the disease does not always stop on its own and may in fact be associated with kidney damage and death.

Eugen Frank In 1915 in Breslau he named this syndrome"anaphylactoid purpura", however, this term is not currently used due to the fact that the allergic nature of the disease has not been confirmed. One of Frank's patients was a 15-year-old girl who had suffered from recurrent purpura for 3 years. Frank noted that the appearance of the rash was provoked by the imposition of a compressive bandage on the limb.

Etiology The etiology of the disease has not been established The list of etiological agents associated with the development of Henoch-Schonlein purpura includes group A β-hemolytic streptococcus, Haemophilus influenzae, chlamydia, mycoplasmas, Legionella, Yersinia, Epstein-Barr, Coxsackie, hepatitis B and C viruses, adenovirus , cytomegalovirus, parvovirus B19, salmonella, Helicobacter pylori, Clostridium difficile There are isolated cases of Henoch-Schonlein purpura, which developed after vaccination against typhoid, measles, influenza

Etiology (continued) In addition to infectious factors, alcohol, drugs, food, hypothermia, insect bites can act as a trigger. Single observations of the development of Henoch-Schonlein purpura in close relatives may indicate a possible role of hereditary predisposition

The pathogenesis of Shenlein-Henoch purpura is considered as an immunocomplex disease associated with deposition in vascular wall and tissues of granular IgA deposits and complement activation. BUT in last years it was shown that: IgA has the ability to reduce the production of pro-inflammatory cytokines (tumor necrosis factors α, interleukin 6) and is not able to activate complement IgA is found in the endothelium of intact vessels and in the mesangium of intact renal glomeruli observed the observation of Henoch-Schonlein purpura with complete selective deficiency of IgA

Pathogenesis suggests the possibility of participation of endotoxin in the development vascular inflammation, mediated by the Schwartzman reaction, dysfunction of the intestinal barrier of the development of transient endotoxemia chronic inflammation intestinal wall due to dysfunction of its local immune system or infectious process Pathologically increased intestinal permeability for macromolecules was found in most patients with Henoch-Schonlein purpura during exacerbations of cutaneous vasculitis. The most likely source of endotoxin is the gastrointestinal tract

Clinical picture The clinical picture of Henoch-Schonlein purpura consists of four typical manifestations: skin hemorrhagic rash 100% articular syndrome 75% abdominal syndrome 65% kidney damage 40% The disease may have an acute onset and be accompanied by general, constitutional symptoms, weakness, malaise, fever Number of organ manifestations Henoch-Schonlein purpura varies from 1-2 to a combination of all 4 classic features that can develop in any sequence over several days or weeks of illness

Skin lesions Skin lesions at various periods of the disease are observed in all patients with Henoch-Schonlein purpura and are an obligatory (sine qua none) criterion for diagnosing skin changes most often manifest as bilateral symmetrical hemorrhagic rash(purpura) with a size of rashes from 3 to 10 mm. At the very beginning of its development, the skin elements of a hemorrhagic rash are papules that rise above the surface of the skin, as a result of which they can be easily felt on palpation. This feature of the hemorrhagic rash in Henoch-Schonlein purpura is associated with its inflammatory origin and is designated by the term "palpable purpura". Within a few hours, the infiltration of the skin rash disappears, hemorrhagic papules transform into hemorrhagic spots and purpura ceases to be palpable; petechiae of pinpoint hemorrhages up to 3 mm in size are also possible; The most typical localization of linear ecchymosis are places subject to increased mechanical compression (skin folds, elastic band of socks, tight belt, tonometer cuff). This phenomenon in Schonlein-Henoch purpura is analogous to the Konchalovsky-Rumpel-Leede symptom or tourniquet symptom.

Skin lesions Skin hemorrhages do not blanch with pressure, which makes it possible to distinguish them from erythema Most typical localization skin rashes lower limbs of the leg and foot. Often, purpura spreads to the thighs, buttocks, torso, upper limbs and extremely rare on the face. In the process of evolution, the rashes gradually turn pale, transform into brown pigment spots and then disappear. With a long recurrent course, the skin at the site of former rashes may become pigmented due to the development of hemosiderosis. A characteristic feature of cutaneous vasculitis in Henoch-Schonlein purpura is a tendency to relapse after long stay patient in an upright position. In severe skin lesions, a fusion of hemorrhagic rashes with their subsequent bullous transformation, ulceration and the formation of long-term healing erosions and ulcers can be observed. Along with a hemorrhagic rash, skin lesions can be represented by erythematous-macular and urticarial elements. In some cases, skin rashes may be accompanied by itching and local swelling of tissues.

Joint damage As a rule, it develops in parallel with skin lesions, usually has the character of migrating polyarthralgia, arthritis is less common, localization of inflammatory changes in the knee and ankle joints, elbow, wrist and other joints are less often affected, these manifestations of the disease are always transient and benign, never lead to the development of persistent changes in joints. The duration of the articular syndrome rarely exceeds 1 week.

The defeat of the gastrointestinal tract is observed in 60-80% of patients of childhood and is often dramatic in nature with the development of severe surgical complications such as intussusception or intestinal perforation. In adult patients, abdominal syndrome develops somewhat less frequently (40–65% of cases) and its course is more favorable. persistent symptom abdominal pain As a rule, abdominal pain occurs suddenly and is cramping in nature, flowing like intestinal colic, which forces the patient to take a forced position with his legs pressed to his stomach. In some cases, eating increases the pain, creating a typical picture of "abdominal toad". Less pain wear aching character and do not significantly violate general condition sick. Anorexia is observed in 70% of patients. The most typical localization of pain is the mesogastric, epigastric or right iliac region. The greatest difficulties for diagnosis are cases that require differential diagnosis with acute appendicitis. Late development of systemic signs of the disease (mainly skin purpura) often causes "unreasonable" appendectomy in such patients

Gastrointestinal tract involvement Clinical signs of peritoneal irritation may occur. In such situations, a thorough examination, including laparoscopy, is necessary to exclude purulent peritonitis as a result of possible perforation of the intestinal wall /or benzidine samples). The severity of bleeding can reach a significant degree and lead to the development of hemorrhagic shock, requiring massive blood transfusions. It should be noted that, in contrast to idiopathic intussusception, which has mainly ileocecal localization, intussusception in Henoch-Schonlein purpura in 70% of cases is detected in the small intestine.

Kidney damage in adults develops almost 2 times more often than in children. In childhood, clinical signs of kidney damage are usually detected during the first month of illness. In 80% of adult patients, involvement in the process of the kidneys is noted in the first 3 months of the disease, however, in chronic recurrent course of cutaneous vasculitis, a delayed onset of signs of nephritis is possible. Possible precursors of involvement in the process of the kidneys in children are: syndrome, persistent cutaneous purpura, and decreased plasma factor XIII levels. In adult patients, risk factors for kidney damage include episodes of infections at the onset of the disease, the presence of fever, the spread of a skin rash on the trunk, severe abdominal manifestations of the disease, and the presence of laboratory signs of inflammatory activity. renal pathology, as a rule, does not correspond to the severity of skin manifestations of the disease. It should be noted that both in children and adults there was a significant positive correlation between the incidence of kidney damage and the development of abdominal syndrome. In children, in half of the cases, kidney damage has a favorable course with complete clinical and laboratory recovery, while in most adult patients there is a tendency to chronic persistent course of nephritis

Kidney damage is represented by glomerulonephritis and in half of the patients it is characterized by mild or moderate urinary syndrome (microhematuria and proteinuria). In a third of patients, macrohematuria is observed, which most often develops at the onset of nephritis, often against the background of respiratory infections, resembling the course of synpharyngitis nephropathy. More severe manifestations of the disease are also possible, including nephrotic and acute nephritic syndrome, rapidly progressive nephritis, and acute renal failure. Hypertension syndrome is found in 14-20% of patients. There is a clear (though not mandatory) relationship between the severity of clinical manifestations of glomerulonephritis and the nature of histological changes in the glomeruli detected by kidney biopsy. So, in patients with asymptomatic hematuria, only a more or less pronounced mesangial proliferation is usually determined. The appearance of proteinuria is accompanied by an increase in cell proliferation and, if proteinuria reaches a nephrotic level, the frequent formation of epithelial "crescents"

Lung damage Single observations in the form of alveolar bleeding are described. Clinical and radiological manifestations of pulmonary vasculitis in Henoch-Schonlein purpura are nonspecific and differ little from those in other systemic vasculitis. In most patients with alveolar bleeding, the prognosis of the disease is extremely unfavorable.

Involvement of the central and peripheral nervous system Clinical manifestations that allow suspecting cerebrovaculitis in the framework of Henoch-Schonlein purpura include persistent headaches, various behavioral disorders, local or generalized convulsive seizures; subarachnoid and intracerebral hemorrhages, subdural hematomas and cerebral infarctions may be the basis of such disorders. that can be detected using computed tomography and magnetic resonance imaging of the brain

Diagnosis is based on the identification of typical clinical signs of the disease, primarily bilateral skin hemorrhagic rashes. The presence of characteristic palpable purpura on the lower extremities in the absence of other systemic manifestations is a sufficient basis for establishing the diagnosis of Henoch-Schonlein purpura, provided that the secondary nature of the hemorrhagic rash is excluded

Diagnostics Laboratory research Changes in clinical analysis blood can reflect the inflammatory activity of the disease (ESR), as well as the severity of complications (anemia in intestinal bleeding). The presence of thrombocytopenia is an exclusion criterion for Henoch-Schonlein purpura. In uncomplicated forms, biochemical tests are uninformative. Disease activity reflects the level of von Willebrand factor and thrombomodulin in blood plasma. It is important to note that detection high level fibrin/fibrinogen degradation products in plasma active forms disease is not a sign of the development of DIC, but only reflects the high inflammatory activity of the disease

Diagnosis Immunological examination should include the study of antistreptolysin O (ASL-O), C-reactive protein (CRP), rheumatoid factor(RF), antinuclear factor (ANF), antibodies to native (double-stranded) DNA, complement, cryoglobulins, cryofibrinogen, antineutrophil cytoplasmic antibodies (ANCA), anti-Ro(SS-A), antibodies to cardiolipin. Most of these indicators are necessary to exclude other diseases that occur with skin purpura. Virological testing (aimed at detecting hepatitis B and C viruses) is indicated for all patients with skin hemorrhagic rashes to exclude skin vasculitis associated with chronic viral diseases Liver Analysis of feces for dysbacteriosis

Instrumental studies Key role in validation clinical diagnosis a biopsy of the skin and / or kidneys plays, less often of other organs, with a mandatory immunohistochemical study. A typical finding in a skin biopsy with light microscopy is a picture of leukocytoclastic vasculitis: fibrinoid necrosis of the vascular wall and perivascular infiltration by neutrophils with their decay and the formation of leukocyte detritus. It should be emphasized that a similar morphological picture is found in all vasculitis occurring with damage to small vessels. A characteristic, but not absolute, sign of Schonlein-Henoch purpura is the fixation of IgA-containing immune complexes in the vascular wall, which are detected by immunohistochemistry. Diagnosis

Diagnosis Instrumental studies Histological examination of the glomeruli is indicated to clarify the long-term prognosis of glomerulonephritis. The morphological picture of kidney damage in Henoch-Schonlein purpura is identical to that in Berger's disease (primary IgA nephropathy). The most common morphological variant of kidney damage is mesangioproliferative glomerulonephritis, characterized by focal or diffuse proliferation of mesangiocytes. Immunohistochemical examination reveals granular deposits of IgA, less often IgG, as well as C3 fractions of complement, fibrin. In more severe cases the formation of epithelial "crescents" is noted

Diagnosis Severe abdominal syndrome may require endoscopic studies, including laparoscopy, to exclude perforation of the wall of the small or large intestine. The development of erosive-hemorrhagic duodenitis with the predominant involvement of the descending part of the duodenum is most typical for the abdominal form of Shenlein-Henoch purpura. Colon involvement is much less common and may manifest as aphthous ulcers and petechial lesions, often localized in the descending colon and sigmoid colon.

Diagnostics In recent years, the use of ultrasonography of the abdominal cavity, which allows to detect: edema of the intestinal wall, hematoma, dilation of various parts of the intestine, and also to assess the extent of these changes. Repeated studies can trace the evolution of intestinal damage in the form of a decrease or increase in edema of the intestine, spontaneous resolution or recurrence of intussusception, a decrease in intestinal dilation and a change in the amount of free fluid

Diagnostics Informative method diagnosis of abdominal lesions is contrast radiography, which reveals in various departments mainly small intestine: thickening of the mucosal folds, motor dysfunction in the form of dilation or spasm, and filling defects ("finger impressions")

Classification criteria (1995) Presence of IgA deposits in skin vessels Age of the patient is less than 20 years Presence of abdominal lesions (abdominalgia or intestinal bleeding) Presence of a respiratory infection at the onset of the disease Demonstration of IgA deposits in the mesangial matrix of the renal glomeruli To establish definite diagnosis Henoch-Schonlein purpura requires at least 3 of the above 5 criteria.

Differential diagnosis Differential diagnosis is carried out with a wide range of diseases that occur with cutaneous leukocytoclastic vasculitis: vasculitis of small vessels (Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome, cryoglobulinemic vasculitis, cryofibrinogenemic vasculitis) vasculitis with autoimmune diseases(systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis, Sjögren's disease, Crohn's disease, ulcerative colitis) vasculitis in infections, malignant neoplasms, drug allergies

Indications for consultation of other specialists All patients are recommended to consult a rheumatologist Given the association of the disease with infections of the mucous membranes, all patients are routinely shown to consult an ENT doctor, urologist, gynecologist kidneys), gastroenterologist (damage to the gastrointestinal tract), cardiologist (damage to the heart), pulmonologist (damage to the lungs), neurologist (damage to the nervous system).

Non-pharmacological treatment Rarely used, mainly as an adjunct drug therapy: with alveolar bleeding, rapidly progressive nephritis and acute renal failure, an exchange transfusion of plasma (plasmapheresis) is indicated. With the development of renal failure, hemodialysis is indicated

Drug treatment In case of skin lesions, colchicine dapsone disulon dimocyfon sulfasalazine combination of dapsone with pentoxifylline glucocorticoids are effective in the vast majority of patients, especially at high doses, but their long-term use in patients with Henoch-Schonlein purpura without involvement of internal organs is a mistake, since the severity of side effects in such a situation may exceed the severity of the disease itself

Drug treatment Prescribing regimens: colchicine 1–2 mg orally once a day, for a long time; dimocyfon inside 100 mg 1 time per day, for a long time; dapsone 100 mg orally once a day, long-term; disulon inside 100 mg 1 time per day, for a long time (the drug is not registered in Russia, which makes it difficult to obtain it); pentoxifylline inside 400 mg 3 times a day, for a long time; sulfasalazine inside 500-1000 mg 2 times a day, for a long time.

Drug treatment Damage to the gastrointestinal tract with intense abdominal pain absolute indication for the appointment of glucocorticoids: prednisone intravenously drip 300-500 mg / day for 3 days in a row, followed by a transition to oral administration of 0.5 mg / kg 1 time per day for 2- 3 weeks, then rapid decline doses of 5 mg every 3 days until complete withdrawal. A contraindication to the appointment of glucocorticoids inside with abdominal syndrome can only be perforation of the intestinal wall.

Drug treatment The greatest problems are most often associated with the choice of treatments chronic glomerulonephritis: Most authors consider it justified to use ultra-high doses of glucocorticoids, cytostatics and / or plasmapheresis sessions in case of severe glomerulonephritis (more than 50% of glomeruli with epithelial "crescents").

Drug treatment In this case, the following scheme is used: oral prednisolone 1 mg / kg 1 time per day for 4-6 weeks, then dose reduction by 2.5 mg / week until completely canceled or until a maintenance dose of 5-10 mg / day is taken or prednisolone intravenously 15 mg/kg once a day for 3 days (total 6–20 three-day "pulses" with an interval of 3 weeks) cyclophosphamide intravenously 15 mg/kg once every 3 weeks, under the control of the level of peripheral blood leukocytes (total 6–20 “pulses”) plasmapheresis with an exfusion volume of 30–60 ml/kg, 10–14 sessions

Medical treatment For less severe forms glomerulonephritis there is no consensus. for the treatment of patients with nephrotic and rapidly progressive glomerulonephritis, it is proposed to use intravenous immunoglobulins: o human normal immunoglobulin intravenously at 400-1000 mg for 1-5 days, repeated courses once a month for 6 months. In uncontrolled trials conducted by French authors, the use of immunoglobulin was associated with a clear improvement in the condition of most patients. The mechanism of action of immunoglobulin is inclined to explain the positive effect of the drug by the dissolution of immune complexes. Unfortunately, the practical use of such therapy is limited by the high cost of treatment.

Drug treatment Correction of hemostasis disorders is currently considered only as an auxiliary method of therapy, the prospects of which are assessed skeptically. At the same time, the use of factor XIII in "abdominal purpura" due to the high cost of the drug, apparently, can be a real alternative to glucocorticoids only in rare cases their inefficiency (or contraindications to their use): clotting factor XIII intravenously 15000–25000 IU for 3–7 days or urokinase intravenously slowly 5000 IU/kg 3 times a week for 3–12 weeks.

Surgical treatment Surgical treatment is carried out in patients with severe lesions of the gastrointestinal tract and the development of surgical complications (invagination or perforation of the intestine). In case of development of terminal renal failure, a kidney transplant can be performed. It is believed that clinically significant recurrences of glomerulonephritis in the transplant occur in approximately 35% of patients and lead to complete loss of function of the transplanted kidney in 10% of cases. Apparently, relapses especially often develop in patients with initially severe glomerulonephritis, when terminal renal failure develops within less than three years from the onset of the disease. Anecdotal observations suggest that the risk of recurrent glomerulonephritis in the graft may be higher in the case of a related transplant. Further management Outpatient observation should be carried out by specialists with experience in the treatment of this disease. Sometimes, even during treatment, it is possible to develop life-threatening conditions that require urgent hospitalization in a multidisciplinary hospital with relevant experience. It is often possible to achieve a complete cessation of treatment. However, it should be remembered that premature discontinuation of treatment can lead to the development of an exacerbation.

Prognosis Despite the fact that the course of Shenlein-Genoch purpura in most patients during the first year of the disease (and even the first five years) seems to be benign, the long-term prognosis is often less favorable. Thus, among patients who fell ill in childhood, after 20 years, a significant clinical deterioration was noted in 22%; moreover, 10% of patients with a “complete” clinical and laboratory remission were registered in the number of patients with a worsening condition. Attention is also drawn to the often complicated course of pregnancy in women who have undergone Henoch-Schonlein purpura: arterial hypertension and proteinuria is observed in 36% of them

Prognosis In a cohort of adults with Henoch-Schonlein purpura who were followed for 15 years, 11% needed replacement therapy program hemodialysis and another 13% developed chronic renal failure with a decrease in glomerular filtration rate less than 30 ml / min. Sclerotic changes in the glomeruli at the initial biopsy are closely related to the poor outcome of glomerulonephritis. It is important that in most patients with late progression of glomerulonephritis there are no signs of clinical activity of renal and extrarenal lesions, which is explained by the predominant influence on the course of the disease of non-immune mechanisms of progression . On the pathogenesis of Henoch-Schonlein purpura. // Doctor S. 17– Mukhin N. A., Gulyaev S. V., Krivosheev O. G., Semenkova E. N. et al. Clinical and prognostic significance of lesions of the gastrointestinal tract in systemic vascular purpura. // Therapeutic archive P. 50 – Rostoker G. Schönlein–Henoch purpura in children and adults: diagnosis, pathophysiology and management. // Biodrugs Vol P. 99–138. (PMID:)

Indications for consultation of other specialists Rheumatologist conducting intensive care, any change in treatment. With exacerbations of infections of the mucous membranes, a consultation with an ENT doctor, urologist, gynecologist is indicated. If the course of organ damage worsens, consultations of a dermatologist (with skin damage), a nephrologist (kidney damage), a gastroenterologist (damage to the gastrointestinal tract), a cardiologist (heart damage), a pulmonologist (lung damage), a neurologist (damage to the nervous system) are possible. With the development of surgical complications, a consultation with the surgeon is necessary.

• Symptoms of Senlein Disease - Henoch
• Which doctors should you contact if you have Senlein-Henoch disease

What is Senlein-Henoch Disease?

Hemorrhagic vasculitis- one of the most common hemorrhagic diseases, which is based on multiple microthrombovasculitis, affecting the vessels of the skin and internal organs. The disease often occurs in childhood and among children under 14 years of age is observed with a frequency of 23-25 ​​per 10,000.

What Causes Senlein Disease?

Currently, hemorrhagic vasculitis has been proven to belong to immunocomplex diseases, in which microvessels undergo aseptic inflammation with more or less deep damage to the walls, thrombosis and the formation of circulating immune complexes (CIC).

Pathogenesis (what happens?) during Senlein-Henoch disease

The reason for the development of this pathology is the formation of circulating immune complexes in the bloodstream. These substances settle on inner surface blood vessels, causing damage to them.

Classification

This handbook provides a classification of hemorrhagic vasculitis by G. A. Lyskina (2000).

1. Form (evolution) of the disease:
   1. initial period;
   2. improvement;
   3. exacerbation.
2. Clinical forms:
   1. simple;
   2. mixed.
3. Clinical Syndromes:
   1. cutaneous;
   2. articular;
   3. abdominal;
   4. renal.
4. Severity.

1. general condition - satisfactory;
2. mild rashes;
3. possible arthralgia.

1. general condition - moderate;
2. profuse rashes;
3. arthralgia, arthritis;
4. recurrent abdominal pain;
5. microhematuria;
6. slight proteinuria (traces of protein in the urine).

1. general condition - severe;
2. plentiful confluent rashes with elements of necrosis;
3. chronic angioedema;
4. persistent pain in the abdomen;
5. gastrointestinal bleeding;
6. macrohematuria;
7. nephrotic syndrome;
8. acute renal failure.

1. acute (up to 2 months);
2. protracted (up to 6 months);
3. chronic.

Clinic

With hemorrhagic vasculitis, the vessels of any area can be affected, including the lungs, the brain and its membranes.

Skin syndrome occurs most frequently. With it, the limbs, buttocks are symmetrically affected, less often the torso. There is a papular-hemorrhagic rash, sometimes with blisters. The rashes are of the same type, at first they have a distinct inflammatory basis, in severe cases they are complicated by central necrosis and become covered with crusts, leaving pigmentation for a long time. When pressed, the elements of the rash do not disappear.

Articular syndrome often occurs together with skin or several hours or days after it in the form of pain of varying intensity in large joints (knee, elbow, hip). After a few days, the pain disappears, but with a new wave of rashes, it may occur again. In some cases, the articular lesion is persistent and persistent, reminiscent of rheumatoid arthritis.

Abdominal syndrome is more often observed in childhood (in 54-72% of patients), in approximately 1/3 it prevails in the clinical picture, in some cases it precedes skin changes, which makes diagnosis very difficult. The main symptom is severe pain in the abdomen, constant or cramping, sometimes so intense that the patient does not find a place in bed and screams for many hours. The pain is due to hemorrhages in the intestinal wall. These hemorrhages can be combined with soaking the intestinal wall and mucous membrane with blood, bleeding from it and from areas of necrosis, hematemesis, chalky (admixture of blood in the feces) or fresh blood in the feces, as well as false urges with frequent stools or, conversely, with its delay. From the very beginning, fever, more or less pronounced leukocytosis (an increase in the number of leukocytes in the blood) are determined. At heavy bleeding collapse develops ( fainting) and acute posthemorrhagic anemia. In some cases frequent vomiting leads to a large loss of fluid and chlorides. In the coagulogram, hyperthrombocytosis and hypercoagulability are determined.

In a significant part of patients, the abdominal syndrome is short-lived and resolves on its own in 2-3 days. Periods severe pain can alternate with painless intervals lasting about 1-3 hours. This helps to distinguish abdominal syndrome from acute surgical diseases of the abdominal organs. Such differentiation is especially difficult in patients without skin-articular manifestations and with symptoms of peritoneal irritation. More often, the abdominal syndrome mimics acute intestinal obstruction (intussusception), appendicitis, torsion and ovarian cysts, and perforation of an intestinal ulcer.

Comparative diagnosis can cause certain difficulties for the doctor - this is due to the fact that hemorrhagic vasculitis itself can cause all of the listed surgical diseases of the abdominal organs. So, for example, many cases of invagination (introduction of one section of the intestine into another) and obstruction of the intestine due to compression or closure of its lumen by a hematoma (especially in children under 2 years old), necrosis of the intestine and its perforation (formation of a through defect), acute appendicitis and other complications requiring surgical intervention. Difficulties in differential diagnosis in similar situation lead to the fact that some patients with hemorrhagic vasculitis undergo unreasonable surgical interventions.

In adult patients, abdominal syndrome is less common and in most cases does not warrant a diagnostic laparotomy, rarely complicated by intestinal obstruction and peritonitis (inflammation of the peritoneum). In the elderly

In age, an abdominal variant of the disease is sometimes observed with indefinite and not always pronounced abdominal pain and persistent intestinal bleeding, the source of which cannot be determined. In search of a malignant neoplasm, a latent ulcer of the intestine or a bleeding polyp in such cases, they often go for a trial laparotomy and a wide examination of the abdominal organs. In the elderly with hemorrhagic vasculitis, such an operation, which does not give any tangible results, usually ends with atony (complete lack of tone) of the intestine and dynamic intestinal obstruction, a sharp increase in general intoxication, the addition of cardiovascular insufficiency and death of the patient. Meanwhile, the correct recognition of Senlein-Henoch disease in such cases or even a trial course of treatment for this disease in diagnostically unclear cases allows you to quickly stop all symptoms and avoid unindicated and dangerous surgical intervention.

Renal syndrome is found in 1 / 8-1 / 2 of patients and often develops as acute or chronic glomerulonephritis - with micro- or macrohematuria (blood in the urine), proteinuria (from 0.33 to 30% of the protein in the urine). Arterial hypertension in this form of kidney pathology is rare. Possible nephrotic syndrome. Kidney damage often does not occur immediately, but 1-4 weeks after the onset of the disease. Signs of nephritis may persist for only a few weeks or months, but there is also a protracted or chronic course of the disease, which dramatically worsens the prognosis. In some patients, kidney damage progresses rapidly with an outcome in uremia in the first 2 years of the disease. In general, kidney damage is a potentially dangerous manifestation of hemorrhagic vasculitis, and therefore the attending physician must carefully monitor urine composition and kidney function throughout the disease.

Much less often, vascular damage to the lungs is detected, sometimes leading to the development of fatal pulmonary hemorrhage. Also, in quite rare cases, a cerebral form of the disease develops, which occurs with headaches, meningeal symptoms(hemorrhages in the membranes of the brain), epileptiform seizures (resembling seizures in epilepsy).

Often there is an increase in temperature (initially up to 38-39 ° C, then subfebrial, i.e. below 38 ° C), a small and intermittent initial leukocytosis, an increase in ESR, an increase in serum globulins, hyperfibrinogenemia (increased fibrinogen in blood plasma) . Due to blood loss, anemia develops.

Diagnosis of Senlein Disease - Henoch

The diagnosis of hemorrhagic vasculitis is based on clinical findings and does not require additional research for confirmation. In the analysis of peripheral blood, leukocytosis of varying severity, increased acceleration of ESR, neutrophilia (increase in the number of neutrophilic leukocytes), eosinophilia (increase in the number of eosinophils), thrombocytosis (increase in the number of platelets) are detected. Given the frequent damage to the kidneys, all patients need to systematically do urine tests. If there are changes in the urine, studies are performed to evaluate functional state kidneys. Due to the fact that 1/3 of patients may have DIC, it is advisable to regularly count the number of platelets, and during the height of the disease to study the state of the patient's hemostasis (venous blood clotting time, heparin resistance, fibrinogen and fibrin levels in the blood).

Timely diagnosis of complications of abdominal syndrome - appendicitis, intussusception, intestinal perforation and peritonitis - causes great difficulties. Such children need joint supervision of a pediatrician and a pediatric surgeon in dynamics.

Treatment of Senlein Disease - Henoch

A prerequisite for therapy is hospitalization and compliance bed rest for at least 3 weeks, then it is gradually expanded, as exacerbations of purpura are possible, explained as orthostatic purpura.

Cooling and additional allergization of patients should be avoided in every possible way. food products And medicines. Cocoa, coffee, chocolate, citrus fruits, fresh berries (strawberries, strawberries) and dishes from them, as well as individually intolerable types of food, are excluded from the diet.

The use of antibiotics, sulfonamides and other allergenic drugs (including all vitamins) that can support hemorrhagic vasculitis or exacerbate it should be avoided. Low allergenic antibiotics (ceporin, rifampicin) are prescribed only for background or concomitant acute infectious diseases (for example, lobar pneumonia). Articular syndrome, fever, leukocytosis and increase in ESR are not an indication for the appointment of antibiotics and other antibacterial drugs, since they are characterized by immune aseptic inflammation.

All patients with hemorrhagic vasculitis are advised to prescribe enterosorbents, for example activated carbon, cholestyramine or polyphepan inside. In addition, gastric drops, antiallergic drugs (antihistamines), calcium pantothenate, rutin, average doses of ascorbic acid are prescribed, and phytotherapy is also used. With all this, the effectiveness of the above drugs in the treatment of this pathology remains very doubtful.

Patients develop abdominal pain that does not go away while taking gastric drops, they resort to the use of drugs with analgesic effects, such as no-shpa, baralgin.

The use of antiplatelet agents, such as chimes, pentoxifylline (trental), is considered justified. The duration of treatment is 3 months. In the moderate course of hemorrhagic vasculitis, it is recommended to use 2 antiplatelet agents, and in case of chronic course of the pathology under consideration - add Plaquinil (Delagil) to therapy. The duration of such therapy can last up to 1 year. It is also recommended to prescribe drugs with a membrane-stabilizing effect (vitamins A, E, dimephosphone).

The high activity of the process with a pronounced abdominal, skin and articular syndrome is an indication for the appointment of a combination the following drugs: prednisolone and heparin. The isolated appointment of prednisolone is dangerous, since it contributes to an increase in blood clotting, and there is always a tendency to develop DIC in this disease (even if there are no clear signs of its presence). Prednisolone is usually prescribed at a dose of 1 mg / kg, and heparin - 200-300 IU / kg per day, divided into 4-6 injections, under the skin of the abdomen. If, against the background of heparin therapy, the venous blood coagulation time continues to be shortened (less than 8 minutes), then the dose can be increased by 1.5 times. Heparin should not be administered 2 or 3 times a day, as this provokes the development of intravascular thrombi. Withdrawal of heparin should be gradual, but by reducing the dose rather than reducing the number of injections. Sometimes, with a stormy clinical picture, one has to resort to infusion therapy, and in this case it is possible to achieve optimal administration of heparin - intravenously drip with its uniform intake into the body during the day.

In severe cases, in addition to heparin therapy and glucocorticoids, 5-8 plasmapheresis sessions are prescribed. The first three sessions of plasmapheresis are carried out daily, the next - 1 time in 3 days. Fresh frozen plasma, albumin solutions, glucose are used as replacement drugs.

Perhaps a combination of pulse therapy with prednisolone (15-20 mg / kg / day for 3 days) and plasmapheresis.

In patients with subacute nephritis or with a rapid course of glomerulonephritis, they resort to the combined appointment of immunosuppressants (azathioprine or cyclophosphamide) with glucocorticoids and heparin, antiplatelet agents (curantil). Cytostatics should not be prescribed only in connection with a protracted or undulating course of the disease. Such patients are recommended to be examined for the presence of helminths, foci of infection, i.e., to look for the cause.

With proper treatment, the abdominal syndrome is usually eliminated most quickly, the intensity of which often decreases within a few hours after intravenous administration heparin. Simple (cutaneous-articular) variants of vasculitis are the most persistent. The periodic appearance of a small number of rash elements on the legs and feet without other symptoms is often not subject to therapy at all. Sometimes they are treated with local applications. These rashes are harmless and disappear spontaneously after a while.

There is evidence of the repeated occurrence of exacerbation of hemorrhagic vasculitis due to psycho-emotional stress, hysterical background, stressful situations. The patient is provided with psychological peace, if necessary, is recommended sedatives and tranquilizers, which increases the effectiveness of complex therapy.

Forecast

It is believed that 60% of patients with hemorrhagic vasculitis recover within a month, and 95% within a year.

Chronic nephritis develops in 1-2% of patients suffering from this pathology. Mortality in hemorrhagic vasculitis is about 3% or even less due to forms with organ complications and cases of chronic nephritis.

Dispensary observation

If there is no kidney damage, the children are under dispensary registration with the local pediatrician for 5 years. Every six months, the child is shown to a dentist, an otorhinolaryngologist for timely diagnosis and treatment of the most common foci of infection. Also regularly examine the feces for helminth eggs. Urine tests are done at least once a quarter and after each acute respiratory infection. Medical exemption from vaccinations is given for 2 years. Planned therapy is not indicated.

Prevention of Senlein Disease - Henoch

In the prevention of exacerbations of the disease, an important role is played by the prevention of exacerbations of a chronic infection, the refusal to take antibiotics and other drugs without good indications (taking tetracyclines, chloramphenicol is especially undesirable), and the exclusion of contact with allergens. Patients are contraindicated in vaccinations and tests with bacterial antigens (for example, tuberculin), since they often cause severe relapses of the disease. Relapses can also provoke cooling, physical exercise, eating disorders, alcohol.

Hemorrhagic vasculitis, or Shenlein-Genoch's disease (purpura), is a systemic vasculitis that affects the vessels of the microvasculature (arterioles, capillaries and postcapillary venules), with a characteristic deposition in their wall of immune deposits, consisting mainly of immunoglobulins A (IgA). Clinically, this disease is manifested primarily by a skin hemorrhagic rash in combination with damage to the joints, gastrointestinal tract and kidneys.

The clinical picture of Henoch-Schonlein purpura consists of four typical manifestations: skin hemorrhagic rash, damage to the joints, gastrointestinal tract and kidneys. The disease can have an acute onset and be accompanied by general, constitutional symptoms - weakness, malaise, fever. In most cases, the disease develops gradually, gradually and does not significantly affect the general condition. As a rule, this variant of the onset of the disease is observed with an isolated skin lesion. The number of organ manifestations of Henoch-Schonlein purpura varies from 1-2 to a combination of all 4 classic signs, which can develop in any sequence over several days or weeks of illness. However, in most cases, the disease begins with a skin hemorrhagic rash. Rarely, other organs, in particular the central nervous system and lungs, may be involved in the process. On average, the frequency of the main clinical manifestations of Henoch-Schonlein purpura is as follows:

skin hemorrhagic rash - 100%;

articular syndrome - 75%;

Abdominal syndrome - 65%;

Kidney damage - 40%.

Skin lesions at various periods of the disease are observed in all patients with Henoch-Schonlein purpura and are a mandatory (sine qua none) diagnostic criterion. In most cases, skin hemorrhagic rash becomes the first clinical manifestation disease, which later joins the defeat of other organs and systems.

Skin changes most often manifest as a bilateral symmetrical hemorrhagic rash (purpura) with a rash size of 3 to 10 mm. At the very beginning of its development, the skin elements of a hemorrhagic rash are papules that rise above the surface of the skin, as a result of which they can be easily felt on palpation. This feature of the hemorrhagic rash in Henoch-Schonlein purpura is associated with its inflammatory origin and is designated by the term "palpable purpura". Within a few hours, the infiltration of the skin rash disappears, hemorrhagic papules transform into hemorrhagic spots, and the purpura ceases to be palpable.

It is also possible to develop petechiae - pinpoint hemorrhages up to 3 mm in size.

Ecchymosis is much less common - large irregularly shaped skin hemorrhages with a diameter of more than 10 mm. The most typical localization of linear ecchymosis are places subject to increased mechanical compression (skin folds, elastic band of socks, tight belt, tonometer cuff). This phenomenon in Schonlein-Genoch purpura is analogous to the Konchalovsky-Rumpel-Leede symptom or tourniquet symptom.

Skin hemorrhages do not blanch with pressure, which makes it possible to distinguish them from erythema. The most typical localization of skin rashes is the lower extremities - shins and feet. Often, purpura spreads to the thighs, buttocks, torso, upper limbs, and extremely rarely to the face. In the process of evolution, the rashes gradually turn pale, transform into brown pigment spots and then disappear. With a long recurrent course, the skin at the site of former rashes may become pigmented due to the development of hemosiderosis. A characteristic feature of cutaneous vasculitis in Henoch-Schonlein purpura is a tendency to recur after a long stay of the patient in an upright position. In severe skin lesions, fusion of hemorrhagic eruptions with their subsequent bullous transformation, ulceration and the formation of long-term healing erosions and ulcers can be observed. Along with a hemorrhagic rash, skin lesions can be represented by erythematous macular and urticarial elements. In some cases, skin rashes may be accompanied by itching and local swelling of the tissues.

As a rule, joint damage develops in parallel with skin damage. In a quarter of cases, articular syndrome precedes the appearance of skin rashes. Joint damage in Henoch-Schonlein purpura usually has the character of migrating polyarthralgia, less often arthritis. The favorite localization of inflammatory changes is the knee and ankle joints, the elbow, wrist and other joints are less often affected. These manifestations of the disease are always transient and benign, never leading to the development of permanent changes in the joints. The duration of the articular syndrome rarely exceeds 1 week.

The defeat of the gastrointestinal tract is observed in 60-80% of pediatric patients and is often dramatic in nature with the development of severe surgical complications, such as intussusception or perforation of the intestine. In adult patients, abdominal syndrome develops somewhat less frequently (40–65% of cases) and its course is more favorable. The most constant symptom of a lesion of the gastrointestinal tract in Henoch-Schonlein purpura is abdominal pain. In most cases, abdominalgia occurs simultaneously or several days after the onset of skin rashes and / or articular syndrome. At the same time, in 14–36% of patients, abdominal pain precedes the development of skin purpura by an average of 2 weeks.

As a rule, abdominal pain occurs suddenly and is cramping in nature, flowing like intestinal colic. Their intensity can be so great that it makes the patient scream loudly or take a forced position with his legs pressed to his stomach. In some cases, eating increases the pain, creating a typical picture of "abdominal toad". Less commonly, the pains are dull or aching in nature and do not significantly affect the general condition of the patient. These manifestations are often accompanied by various dyspeptic symptoms due to impaired intestinal motility (nausea, vomiting, diarrhea, less often constipation). Anorexia is observed in 70% of patients. The most typical localization of pain is mesogastric, epigastric or right iliac region. Somewhat less often, pain occurs in the right hypochondrium or is diffuse. The greatest difficulties for diagnosis are cases that occur with pain in the right iliac region and require differential diagnosis with acute appendicitis. Late development of systemic signs of the disease (mainly skin purpura) often causes "unreasonable" appendectomy in such patients.

Perhaps the appearance of clinical signs of irritation of the peritoneum, which in patients with Henoch-Schonlein purpura can often be associated with aseptic peritonitis due to vasculitis of small vessels of the peritoneum. In such situations, a thorough examination, including laparoscopy, is necessary to exclude purulent peritonitis as a result of possible perforation of the intestinal wall.

A frequent complication of abdominal lesions in Henoch-Schonlein purpura is intestinal bleeding, which is clinically bright (melena, hematemesis, hematochezia), or latent (positive guaiac and/or benzidine tests). According to some authors, the frequency of bleeding in abdominal syndrome reaches 35%. As a rule, signs of intestinal bleeding are combined with abdominal pain, although in some cases they are found even in the absence of any subjective signs of damage to the gastrointestinal tract. The severity of bleeding can reach a significant degree and lead to the development of hemorrhagic shock, requiring massive blood transfusions.

Relatively rare complications of abdominal syndrome include:

small bowel obstruction (usually associated with intestinal intussusception);

paralytic ileus;

· pancreatitis;

· appendicitis;

· cholecystitis;

late ileal strictures;

Malabsorption syndrome due to exudative enteropathy.

It should be noted that, in contrast to idiopathic intussusception, which has mainly ileocecal localization, intussusception in Henoch-Schonlein purpura is detected in the small intestine in 70% of cases.

In recent years, it has been established that in most patients with Henoch-Schonlein purpura, subclinical intestinal damage is observed, manifested by a violation of the barrier function of the small intestine for various macromolecules. It has been shown that this barrier dysfunction is based on chronic nonspecific inflammatory process in the mucous membrane of the small intestine, the severity of which correlates with intestinal permeability and the clinical activity of cutaneous vasculitis.

Kidney damage in adults develops almost 2 times more often than in children. In childhood, clinical signs of kidney damage are usually detected during the first month of illness. In 80% of adult patients, the involvement of the kidneys in the process is noted in the first 3 months of the disease, however, in chronic recurrent cutaneous vasculitis, a delayed onset of signs of nephritis is possible - several months or even years after the onset of the disease. Possible precursors to kidney involvement in children are: male gender, age over 5 years, abdominal syndrome, persistent cutaneous purpura, and decreased plasma factor XIII levels. In adult patients, risk factors for kidney damage include episodes of infections at the onset of the disease, the presence of fever, the spread of a skin rash on the trunk, pronounced abdominal manifestations of the disease, and the presence of laboratory signs of inflammatory activity. The severity of renal pathology, as a rule, does not correspond to the severity of skin manifestations of the disease: kidney damage can develop in patients with a single episode of skin hemorrhagic rashes and, at the same time, be absent in continuously recurrent erosive and ulcerative skin lesions. It should be noted that both in children and adults there was a significant positive correlation between the incidence of kidney damage and the development of abdominal syndrome. In children, in half of the cases, kidney damage has a favorable course with complete clinical and laboratory recovery, while in most adult patients there is a tendency to chronic persistent course of nephritis.

Kidney damage in Henoch-Schonlein purpura is represented by glomerulonephritis and in half of the patients it is characterized by mild or moderate urinary syndrome (microhematuria and proteinuria). In a third of patients, gross hematuria is observed, which most often develops at the onset of nephritis, but can also occur at later stages of renal damage, often against the background of respiratory infections, resembling the course of synpharyngitis nephropathy. More severe manifestations of the disease are also possible, including nephrotic and acute nephritic syndrome, rapidly progressive nephritis, and acute renal failure. In 14–20% of patients, a syndrome of arterial hypertension is found.

There is a clear (though not obligatory) relationship between the severity of clinical manifestations of glomerulonephritis and the nature of histological changes in the glomeruli detected by kidney biopsy. So, in patients with asymptomatic hematuria, only a more or less pronounced mesangial proliferation is usually determined. The appearance of proteinuria is accompanied by an increase in cell proliferation and, if proteinuria reaches a nephrotic level, the frequent formation of epithelial "crescents". In patients with recurrent cutaneous purpura and repeated episodes of gross hematuria, the severity of histological changes in the glomeruli may increase. The most important prognostic factor is the proportion of renal glomeruli with "crescents" of the total number of glomeruli. So, according to French authors, who base their conclusions on 151 observations with an assessment of the condition of patients over a period of 1 to 18 years, among patients with more than 50% of "crescents", 37% developed terminal renal failure, and another 18% developed glomerulonephritis. had a chronic progressive course. On the other hand, 85% of patients who reached ESRD had crescents in more than half of the glomeruli. In 70% of patients with full recovery or persistence of minimal changes in the urine "crescents" in the glomeruli were not found.

Isolated observations of lung damage in the form of alveolar bleeding are described. Clinical and radiological manifestations of pulmonary vasculitis in Henoch-Schonlein purpura are nonspecific and differ little from those in other systemic vasculitis. In most patients with alveolar bleeding, the prognosis of the disease is extremely unfavorable.

In rare cases, the development of heart damage is noted: pericarditis with the development of cardiac tamponade, coronaritis with the development of myocardial infarction and congestive heart failure.

Involvement of the central and peripheral nervous system is possible. Clinical manifestations that allow suspecting cerebrovaculitis in the framework of Henoch-Schonlein purpura include persistent headaches, various behavioral disorders, local or generalized convulsive seizures. Such disorders may be based on subarachnoid and intracerebral hemorrhages, subdural hematomas and cerebral infarctions, which can be detected using computed tomography and magnetic resonance imaging of the brain. Compression of brain structures by a hematoma can cause the development of focal neurological symptoms, which can also be observed when small vessels of peripheral or intracranial nerves are affected.

Diagnosis of Henoch-Schonlein purpura is based on the identification of typical clinical signs of the disease, primarily bilateral skin hemorrhagic rashes. The presence of characteristic palpable purpura on the lower extremities in the absence of other systemic manifestations is a sufficient basis for establishing the diagnosis of Henoch-Schonlein purpura, provided that the secondary nature of the hemorrhagic rash is excluded.

Laboratory research

There are no specific laboratory tests for Henoch-Schonlein purpura.

A standard examination includes a clinical blood test with a mandatory assessment of the platelet count; general urine analysis; biochemical analysis blood; electrophoresis of blood serum proteins; coagulogram with evaluation of platelet function.

Changes in the clinical analysis of blood may reflect the inflammatory activity of the disease (ESR), as well as the severity of complications (anemia in intestinal bleeding). The presence of thrombocytopenia is an exclusion criterion for Henoch-Schonlein purpura.

In uncomplicated forms of Shenlein-Henoch purpura, biochemical tests are uninformative, but can help in differential diagnosis cutaneous leukocytoclastic vasculitis (cytolysis syndrome in vasculitis associated with hepatitis viruses; hyperproteinemia in multiple myeloma and Sjögren's disease). A pronounced increase in ESR and significant dysproteinemia are not typical for Henoch-Schonlein purpura.

Disease activity reflects the level of von Willebrand factor and thrombomodulin in plasma. It is important to take into account that the detection of a high level of fibrin/fibrinogen degradation products in plasma in active forms of the disease is not a sign of the development of DIC, but only reflects the high inflammatory activity of the disease.

Immunological study should include the study of antistreptolysin O (ASL-O), C-reactive protein (CRP), rheumatoid factor (RF), antinuclear factor (ANF), antibodies to native (double-stranded) DNA, complement, cryoglobulins, cryofibrinogen, antineutrophil cytoplasmic antibodies (ANCA), anti-Ro(SS-A), antibodies to cardiolipin. Most of these indicators are necessary to exclude other diseases that occur with skin purpura.

A virological study (aimed at detecting hepatitis B and C viruses) is indicated for all patients with skin hemorrhagic rashes to exclude skin vasculitis associated with chronic viral liver diseases.

Analysis of feces for dysbacteriosis.

Instrumental Research

A key role in confirming the clinical diagnosis is played by a biopsy of the skin and / or kidneys, less often of other organs, with the obligatory immunohistochemical study.

A typical finding in a skin biopsy with light microscopy is a picture of leukocytoclastic vasculitis: fibrinoid necrosis of the vascular wall and perivascular infiltration by neutrophils with their decay and the formation of leukocyte detritus. It should be emphasized that a similar morphological picture is found in all vasculitis occurring with damage to small vessels. A characteristic, but not absolute, sign of Schonlein-Henoch purpura is the fixation of IgA-containing immune complexes in the vascular wall, which are detected by immunohistochemistry. In addition to Henoch-Schonlein purpura, IgA deposits can be detected in skin lesions within chronic inflammatory diseases bowel (Crohn's disease, ulcerative colitis), chronic diffuse liver diseases of alcoholic etiology, celiac disease, Dühring's herpetiform dermatitis.

Histological examination of the renal glomeruli is indicated to clarify the long-term prognosis of glomerulonephritis. The morphological picture of kidney damage in Henoch-Schonlein purpura is identical to that in Berger's disease (primary IgA nephropathy). The most common morphological variant of kidney damage is mesangioproliferative glomerulonephritis, characterized by focal or diffuse proliferation of mesangiocytes. Immunohistochemical examination reveals granular deposits of IgA, less often IgG, as well as C3 fractions of complement, fibrin. In more severe cases, the formation of epithelial "crescents" is noted.

Severe abdominal syndrome may require endoscopic examinations, including laparoscopy, to exclude perforation of the wall of the small or large intestine. The development of erosive-hemorrhagic duodenitis with the predominant involvement of the descending part of the duodenum is most typical for the abdominal form of Shenlein-Genoch purpura. Colon involvement is much less common and may manifest as aphthous ulcers and petechial lesions, often localized in the descending colon and sigmoid colon.

In recent years, ultrasound examination of the abdominal cavity has been increasingly used, which makes it possible to detect edema of the intestinal wall, hematoma, dilation of various parts of the intestine, and also to assess the extent of these changes. With repeated studies, it is possible to trace the evolution of intestinal damage in the form of a decrease or increase in intestinal edema, spontaneous resolution or recurrence of intussusception, a decrease in intestinal dilatation and a change in the amount of free fluid.

An informative method for diagnosing abdominal lesions is contrast radiography, which reveals thickening of the mucosal folds in various parts of the predominantly small intestine, motor dysfunction in the form of dilation or spasm, and filling defects. The latter were called "finger impressions" due to a clear-shaped deformation of the internal contour of the intestine, which occurs against the background of a sharp edema and hemorrhages in the intestinal wall.

Treatment Goals

· Increasing the duration and quality of life.

Achievement of remission.

Reducing the risk of exacerbations.

Prevention of irreversible damage to vital organs.

Indications for hospitalization

· For the first time detected Henoch-Schonlein purpura to clarify the diagnosis and selection of treatment.

Exacerbation of the disease.

development of complications.

Non-drug treatment

It is rarely used, mainly as an addition to drug therapy: in case of alveolar bleeding, rapidly progressive nephritis and acute renal failure, an exchange transfusion of plasma (plasmapheresis) is indicated with an exfusion of 30-60 ml / kg per session and replacement with donor plasma or 5% albumin solution. With the development of renal failure, hemodialysis is indicated.

Medical treatment

For skin lesions, drugs such as colchicine, dapsone, disulon, dimocyfon, sulfasalazine, a combination of dapsone with pentoxifylline can be effective. The mechanism of action of sulfasalazine seems to be associated with the suppression of intestinal inflammation and the normalization of mucosal permeability. Glucocorticoids are effective in the vast majority of patients, especially in high doses, but their long-term use in patients with Henoch-Schonlein purpura without involvement of internal organs is a mistake, since the severity of side effects in such a situation may exceed the severity of the disease itself.

Prescribing regimens:

· colchicine inside 1-2 mg 1 time per day, for a long time;

Dimocyfon inside 100 mg 1 time per day, for a long time;

dapsone 100 mg orally once a day, long-term;

· disulon inside 100 mg 1 time per day, for a long time (the drug is not registered in Russia, which makes it difficult to obtain);

Pentoxifylline 400 mg orally 3 times a day for a long time;

sulfasalazine inside 500-1000 mg 2 times a day, for a long time.

The defeat of the gastrointestinal tract with intense abdominal pain is an absolute indication for the appointment of glucocorticoids:

prednisolone intravenously drip 300-500 mg / day for 3 consecutive days, followed by a transition to oral administration of 0.5 mg / kg 1 time per day for 2-3 weeks, then a rapid dose reduction of 5 mg every 3 days until complete withdrawal.

It is important that gastrointestinal bleeding (in the event that it is due to vasculitis, and not other reasons) is not a contraindication to the appointment of glucocorticoids inside, but, on the contrary, is one of the main indications for such treatment. Withdrawal of glucocorticoid use in severe abdominal syndrome (“motivated”, for example, by the presence of signs gastrointestinal bleeding) is deeply erroneous, since alternative methods treatment of abdominal lesions in Henoch-Schonlein purpura are significantly inferior to glucocorticoids in their effectiveness. A contraindication to the appointment of glucocorticoids inside with abdominal syndrome can only be perforation of the intestinal wall, which currently rarely complicates the course of Henoch-Schonlein purpura.

The greatest problems are most often associated with the choice of treatments for chronic glomerulonephritis: underestimation of the severity of kidney damage and failure to timely conduct active immunosuppressive therapy can lead to the development of treatment-resistant glomerulonephritis, including rapidly progressive; on the other hand, the unjustified prescription of highly toxic cytostatic drugs for benign forms of kidney damage is also unacceptable. A kidney biopsy can reduce the degree of uncertainty in such situations. Most authors consider it justified to use ultra-high doses of glucocorticoids, cytostatics and / or plasmapheresis sessions in case of severe glomerulonephritis (more than 50% of glomeruli with epithelial "crescents").

In this case, the following scheme is used:

oral prednisolone 1 mg/kg once a day for 4–6 weeks, then dose reduction by 2.5 mg/week until complete withdrawal or maintenance dose of 5–10 mg/day or intravenous prednisolone 15 mg/kg once per day for 3 days (total 6-20 three-day "pulses" with an interval of 3 weeks)

cyclophosphamide intravenously 15 mg/kg once every 3 weeks, under the control of the level of peripheral blood leukocytes (total 6-20 "pulses")

plasmapheresis with an exfusion volume of 30–60 ml/kg, 10–14 sessions.

With regard to less severe forms of glomerulonephritis, there is no consensus. In IgA nephropathy (but not in Henoch-Schonlein purpura), in 3 randomized trials with a control group of patients, the effectiveness of cyclophosphamide (in combination with dipyridamole and warfarin) was demonstrated, which consisted in reducing proteinuria, stabilizing kidney function and inhibiting the development of glomerulosclerosis. However, the possibility of widespread use of this drug is limited by its high toxicity.

In addition, for the treatment of patients with nephrotic and rapidly progressive glomerulonephritis, it is proposed to use intravenous immunoglobulins:

normal human immunoglobulin intravenously 400-1000 mg for 1-5 days, repeated courses once a month for 6 months.

In uncontrolled trials conducted by French authors, the use of immunoglobulin was associated with a clear improvement in the condition of most patients. The mechanism of action of immunoglobulin used at high doses for intravenous administration or at medium doses for intramuscular administration remains unclear; the authors tend to explain the positive effect of the drug by the dissolution of immune complexes. Unfortunately, the practical use of such therapy is limited by the high cost of treatment.

Correction of hemostasis disorders, previously considered priority in the treatment of Henoch-Schonlein purpura, is currently considered only as an auxiliary method of therapy, the prospects of which are skeptical. IN in practical terms two recent reports by Japanese researchers are of interest: successful treatment of severe abdominal syndrome in Henoch-Schonlein purpura with infusions of blood coagulation factor XIII (fibrin-stabilizing) and on the favorable long-term clinical and pathomorphological effect of fibrinolytic urokinase therapy on the course of glomerulonephritis in Henoch-Schonlein purpura. At the same time, the use of factor XIII in "abdominal purpura" due to the high cost of the drug, apparently, can be a real alternative to glucocorticoids only in rare cases of their inefficiency (or contraindications to their use):

coagulation factor XIII intravenously 15,000–25,000 IU for 3–7 days

urokinase intravenously slowly 5000 IU/kg 3 times a week for 3-12 weeks.

Surgery

Surgical treatment is carried out in patients with severe lesions of the gastrointestinal tract and the development of surgical complications (invagination or perforation of the intestine).

Hemorrhagic vasculitis (Schonlein-Genoch disease) is the most common vasculitis in children, occurring with damage to the skin, joints and internal organs (mainly the gastrointestinal tract and kidneys).

The disease is registered in children aged 6 months to 18 years, and more often in boys. The annual incidence is 13.5-18 cases per 100 thousand children. In 50% of cases, Henoch-Schonlein disease (HSP) develops in children older than 5 years and in 75% of cases in children older than 10 years. However, at present, cases of HCG disease are often observed among the adult population.

Syndromes of acute purpura and arthritis in children were first described by J. L. Schonlein in 1837, E. Henoch in 1874 added clinical picture diseases intestinal colic and jade.

In the early 1950s, P. M. Zeek published several reports of an acute form of systemic arteritis in adults, which proceeded with purpura-type skin lesions, arthritis, acute abdominal pain, and glomerulonephritis. In most cases, the disease developed while taking sulfa drugs or after vaccination.

The incidence of HSP has a seasonality, its peak falls on the winter period, which made it possible for a number of authors to suggest that the disease is related to streptococcal pharyngitis. However, at present this fact is considered not so obvious.

Pathomorphology. There is acute vasculitis with damage to arterioles and venules in the upper layers of the dermis and intestines. Immunofluorescent examination reveals deposits of immunoglobulins and complement in the vessels of the skin and kidneys, and IgA may be the only one among immunoglobulins. The spectrum of renal pathology is quite wide: from focal focal glomerulitis to necrotizing proliferative glomerulonephritis.

Clinical picture

Fever and skin purpura are the first signs of the disease. The rash is localized mainly on the limbs, buttocks, lower half of the body, is symmetrical; it is hemorrhagic dots or spots that rise above the surface of the skin (Fig. 8.4, see colors, insert). When pressed, the elements of the rash do not disappear. In some cases, the appearance of new rashes is accompanied by slight itching. After a few days, the purpura turns pale, leaving behind areas of hyperpigmentation. Rash elements never appear on the skin of the face.

Rice. 8.4. Confluent cutaneous purpura

Abdominal syndrome is observed in more than half of patients with HSP, and in children somewhat more often, and is due to the presence of hemorrhage in the intestinal wall, mesentery, and peritoneum. Abdominal pain usually occurs acutely, is localized in the umbilical region, is permanent, can be so pronounced in intensity that patients do not find a place for themselves, lie in bed in a forced position with their legs brought to the stomach. The pain syndrome can recur and intensify after eating, then it becomes dull in nature without a clear localization. As a rule, in the presence of massive rashes, symptoms of peritoneal irritation (peritonism) appear. In these cases, it is difficult to diagnose possible complications (internal gastrointestinal bleeding, dynamic intestinal obstruction).

Kidney damage is the third characteristic syndrome of HSP. The frequency of kidney involvement in the pathological process, according to various researchers, ranges from 20 to 70%, and in a number of cases, nephropathy is detected in 85-100% of patients, and in 80% of patients the symptoms of nephritis develop in the first 4 weeks from the onset of the disease. Kidney damage is characterized by the development of glomerulonephritis. The main clinical manifestation is hematuria, the severity of which does not indicate the severity of organ damage.

Glomerulonephritis in HSP is relatively benign, but in 10–20% of cases in older children and adults it progresses and acquires a chronic course, despite a decrease in other manifestations of the disease. Approximately 10% of patients develop chronic renal failure. High secondary arterial hypertension is not typical for HSP and is detected in about 17% of cases.

Lung involvement is rare. It is caused by capillaritis of the interalveolar septa with hemorrhages into the alveoli. Pulmonary syndrome manifests itself as a cough with a scanty amount of sputum, sometimes hemoptysis, shortness of breath of varying severity. Signs of respiratory failure progress rapidly (within a few days). Auscultation draws attention to a sharp weakening of breathing, moist, muffled rales, crepitus can be heard. At x-ray examination find multiple infiltrates mainly in the middle and lower lobes.

Heart lesions are rare and are caused by the development of coronaritis against the background of other systemic manifestations of CSP. The ECG shows signs of myocardial ischemia in the relevant areas.

CNS involvement was observed in 1-8% of patients. The degree of severity of neurological symptoms is different: from minor headaches to the development of hemorrhagic strokes and edema of the substance of the brain or meninges with the development of meningeal signs and epileptiform seizures.

Distinguish with the flow the following forms HSP: fulminant, acute, subacute (or recurrent) and chronic.
The fulminant form is characterized by an acute onset with the development of all major clinical symptoms. The death of the patient occurs within a few days, in most cases from thrombohemorrhagic complications.
The acute form of HSP, in contrast to the fulminant form, ends in recovery within a few months or acquires a relapsing course.

In the subacute course, systemic manifestations are formed during the first or second year of the disease, repeated exacerbations of varying degrees of activity are noted. Such exacerbations are repeated at different intervals, ranging from several months to six months.

In a chronic course, long-term remissions are observed, lasting for several years.

Laboratory diagnosis can only indicate the severity of the disease. A simple or skin-articular form of the disease can occur without noticeable changes in ESR and acute phase parameters. The prevalence of vascular damage can be indicated by such an indicator as the von Willebrand factor, since the vascular endothelium is the only site of its synthesis.

There may be an increase in circulating immune complexes in the blood of patients, but there is no correspondence between the severity of the disease and the degree of their increase. At the same time, a fairly characteristic immunological manifestation of the disease is an increase in the level of IgA or immune complexes containing IgA, as well as the presence of IgA antineutrophil cytoplasmic or antiendothelial antibodies. Deposits of IgA can be found in the histological material of the skin and glomeruli of the kidneys.

Laboratory tests are of particular importance for the diagnosis of DIC and the selection of adequate heparin therapy.
Approximately 30% of patients with CSP develop the disease at age 20 or older, in which case a kidney biopsy may be useful for diagnostic purposes in adults. According to experts from the American College of Rheumatology, in patients with cutaneous purpura over the age of 20 years, nephrobiopsy data made it possible to exclude HSP in 50% of cases. With nephrobiopsy, granulocytes are found in the vessel wall or perivascularly. Venules are affected more often than arterioles (35-50% and 27-41%, respectively). Mononuclear cells and eosinophils are observed in approximately 14% of cases. Large vessels are not involved in the pathological process.

Classification criteria for hemorrhagic vasculitis are presented in Table. 8.9.

Table 8.9. Classification criteria for hemorrhagic vasculitis adopted by the American College of Rheumatology (1990)

Approximate diagnosis. Hemorrhagic vasculitis, skin-visceral form with lesions of the skin (skin purpura), joints, kidneys (mesangial proliferative glomerulonephritis), subacute course, grade II activity.

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