Schoenlein-Genoch disease and kidney damage

Henoch-Schonlein purpura disease is a systemic vasculitis that affects mainly small vessels with the deposition of immune complexes containing IgA in their walls, and manifests itself in skin lesions combined with lesions of the gastrointestinal tract, kidney glomeruli, and joints. The name "Henoch-Schonlein purpura disease" has several synonyms: anaphylactoid purpura, allergic vasculitis, leukocytoclastic vasculitis, rheumatoid purpura. In Russia, the most common term is "hemorrhagic vasculitis".

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Epidemiology

The disease Schonlein-Henoch purpura was described in 1838 by Schonlein as a combination of arthritis and palpable purpura. In 1868, Henoch described 4 children with gastrointestinal tract lesions in the presence of skin and joint processes, and 30 years later he reported the possibility of kidney damage in this syndrome.

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

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Causes Schoenlein-Genoch disease

Causes of Schonlein-Tenoch purpura are associated with infections, food allergies, drug intolerance, and alcohol consumption. In most cases, the disease is preceded by a nasopharyngeal or intestinal infection.

The development of hemorrhagic vasculitis is associated with a number of bacteria and viruses. The most clearly traced connection of the disease is with an infection caused by streptococci and staphylococci, cytomegalovirus, parvovirus B19, human immunodeficiency virus. Less often, an association is noted with intestinal bacteria, yersinia, mycoplasma.

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

Symptoms Schoenlein-Genoch disease

Hemorrhagic vasculitis is in most cases a benign disease, prone to spontaneous remission or recovery within a few weeks from the moment of its onset. However, in some patients, mainly adults, Henoch-Schonlein purpura acquires a relapsing course with the development of severe kidney damage.

The characteristic extrarenal symptoms of Henoch-Schonlein purpura (skin, joint, and gastrointestinal lesions) may appear in any order over several days, weeks, or simultaneously.

Diagnostics Schoenlein-Genoch disease

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

Most patients with high vasculitis activity have an increase in ESR. In children, 30% of cases show an increase in antistreptolysin-O titers, rheumatoid factor, and an increase in C-reactive protein.

The main laboratory sign of Henoch-Schonlein purpura - an elevated level of IgA in the 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 normalizes in the absence of a relapse 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.

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Treatment Schoenlein-Genoch disease

Treatment of Henoch-Schonlein purpura depends on the predominant clinical symptoms.

• If infection is present, antibacterial therapy is indicated.
• Skin and joint syndromes without visceral manifestations are an indication for the use of NSAIDs.
• In case of severe skin and gastrointestinal lesions, glucocorticoids are prescribed. According to some authors, early administration of prednisolone in a short course prevents the development of glomerulonephritis in Henoch-Schonlein purpura.

Forecast

The prognosis of Henoch-Schonlein purpura is generally favorable, despite 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 that is the main cause of death in patients with Henoch-Schonlein purpura. In Europe, among the causes of terminal chronic renal failure in children, the proportion of nephritis in Henoch-Schonlein purpura exceeds 3%.

The course of glomerulonephritis in hemorrhagic vasculitis in adults and children differs significantly. Children are more likely to have transient hematuria without significant proteinuria and functional disorders. Adults are more likely to have rapidly progressing glomerulonephritis, arterial hypertension, nephrotic syndrome, and early renal dysfunction, which prompts the use of more aggressive therapeutic regimens. In the presence of proteinuria over 1 g/day and/or renal dysfunction, the risk of developing chronic renal failure in children is 18%, and in adults - 28%.

The prognostic value for nephritis in patients with Henoch-Schonlein purpura is not only age, but also clinical manifestations and morphological signs.

Isolated microhematuria is associated with 100% 10-year survival. Proteinuria exceeding 1 g/day, nephrotic or acute nephritic syndromes at the onset of glomerulonephritis worsen the prognosis. Macrohematuria is associated with a high probability of a large percentage of crescents in the renal biopsy and rapid deterioration of renal function.

Of the morphological features, the percentage of glomeruli with crescents and interstitial fibrosis have prognostic significance. In this case, in adult patients with the presence of crescents in less than 50% of glomeruli, the risk of developing chronic renal failure is higher than in children.

In general, Henoch-Schonlein purpura and associated nephritis have a relatively favorable outcome: in the general population of sick children, complete recovery is observed in 94% of cases, and in adults - in 89%.

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