Shenlaine-Henoch disease and kidney damage

Illness of purpura Schonlein-Genocha is a systemic vasculitis that affects predominantly small vessels with the deposition of immune complexes that contain IgA in their wall, and is manifested by skin lesions in combination with gastrointestinal lesions, glomeruli of the kidneys and joints. The name "Schönlein-Henoch purpura disease" has several synonyms: anaphylactoid purpura, allergic vasculitis, leukocytoclastic vasculitis, rheumatoid purpura. The term "hemorrhagic vasculitis" is the most common in Russia.

[1], [2], [3], [4]

Epidemiology

The disease of Shonlein-Henoch purpura is described in 1838 by Schonlein as a combination of arthritis and palpable purpura. In 1868, Henoch described four children with lesions of the gastrointestinal tract in the presence of cutaneous and articular processes, and 30 years later he also reported on the possibility of kidney damage in this syndrome.

Hemorrhagic vasculitis is the most common form of systemic vasculitis. The disease of Shonlein-Genoch purpura occurs in people of any age, but most often in children from 3 to 15 years. In Europe, the incidence of Shonlein-Genocha purpura among children under 14 years is 14 cases per 100 000 children, in the US - 10 cases per 100 000. Boys are 2 times more likely than girls, but with age, the difference in incidence disappears. The disease is more often recorded in the winter months. The kidney damage on average is observed in 25-30% of patients with purple Shonlein-Genoch (from 10-20% in Italy to 50-60% in Austria, the USA, Poland).

[5], [6], [7], [8], [9], [10], [11], [12], [13]

Causes of the shenlen-Henoch disease

The causes of purple Shonlein-Tenoch are associated with infections, food allergies, drug intolerance, and alcohol consumption. In most cases, the disease is preceded by nasopharyngeal or intestinal infection.

The development of  hemorrhagic vasculitis is  associated with a number of bacteria and viruses. The relationship of the disease with infection caused by streptococci  and staphylococcus, cytomegalovirus, parvovirus B19, human immunodeficiency virus was most clearly traced  . Less often note the association with bacteria of the intestinal group, iersinia, mycoplasmas.

The development of the purpura of Schonlein-Henoch disease after the use of certain drugs, including vaccines and serums, antibiotics (penicillin), thiazide diuretics, quinidine, is described.

Symptoms of the shenlen-Henoch disease

Hemorrhagic vasculitis is in most cases a benign disease prone to spontaneous remissions or cure within a few weeks from the time of onset. However, in some patients, predominantly adults, the disease of purple Shonlein-Genocha acquires a recurrent course with the development of severe kidney damage.

Typical extrarenal symptoms are the purpura of Shonlein-Genoch (skin, joint, GI tract) can appear in any sequence for several days, weeks or at the same time.

Diagnostics of the shenlen-Henoch disease

Diagnostics of purple Shonlein-Genocha does not have specific laboratory tests.

In most patients with high vasculitis activity there is an increase in ESR. In children, in 30% of cases, an increase in the titer of antistreptolysin-0, a rheumatoid factor, an increase in the content of C-reactive protein is detected.

The main laboratory indicator of purple Shonlein-Genoch - elevated IgA level in blood plasma - is detected in the acute stage of the disease in 50-70% of patients. A year after the acute episode, the IgA content in most cases is normalized in the absence of recurrence of purpura, even if the urinary syndrome persists. In a third of patients, IgA-containing immune complexes are detected at the time of high vasculitis activity.

[14], [15]

Treatment of the shenlen-Henoch disease

Treatment of purpura Schonlein-Genocha depends on the prevailing clinical symptoms.

• If there is an infection, antibiotic therapy is indicated.
• Skin and joint syndromes without visceral manifestations are an indication for the administration of NSAIDs.
• In severe skin and GI traction, glucocorticoids are prescribed. According to some authors, the early administration of prednisolone in a short course prevents the development of glomerulonephritis in schoonlein-Henoch purpura disease.

Forecast

The prognosis of the purulent disease of Shonlein-Genocha is generally favorable, even in spite of frequent relapses of the disease, observed in almost 50% of patients. The development of glomerulonephritis worsens the prognosis of patients with hemorrhagic vasculitis. It is nephritis with the development of chronic renal failure is the main cause of death of patients with purple Shonlein-Genoch. In Europe, among the causes of terminal chronic renal failure in children, the share of nephritis in schoonlein-Henoch purpura disease is more than 3%.

The nature of the course of glomerulonephritis in hemorrhagic vasculitis varies considerably among adults and children. For children, the presence of transient hematuria is more common without pronounced proteinuria and functional disorders. In adults, faster-progressive glomerulonephritis, arterial hypertension, nephrotic syndrome, early impairment of renal function are more often noted, prompting the appointment of more aggressive therapeutic regimens. In the presence of proteinuria more than 1 g / day and / or renal dysfunction, the risk of developing chronic renal failure in children is 18%, and in adults, 28%.

Prognostic value in nephritis in patients with purple Shonlein-Genoch has not only age, but also clinical manifestations and morphological features.

Isolated microhematuria is associated with 100% 10-year survival. Proteinuria exceeding 1 g / day, nephrotic or acute syndrome in the onset of glomerulonephritis worsen the prognosis. Macrogematuria is associated with a high probability of a large percentage of the half-moon in the kidney biopsy and a rapid deterioration in kidney function.

Of the morphological signs prognostic value is the percentage of glomeruli with semilunar and interstitial fibrosis. In adult patients with the presence of half-moons in less than 50% of the glomeruli, the risk of developing chronic renal failure is higher than in children.

In general, the disease of purple Shonlein-Genoch and related nephritis has a relatively favorable outcome: in the general population of sick children, complete recovery is noted in 94% of cases, adults - in 89%.

[16], [17],