Henoch-Schönlein purpura – old disease, new diagnostic challenges
1 Oddział Chorób Dziecięcych i Noworodkowych, CSK MSW w Warszawie. Ordynator: dr n. med. Adam J. Sybilski
2 Zakład Profilaktyki Zagrożeń Środowiskowych i Alergologii, Wydział Nauki o Zdrowiu WUM w Warszawie. Kierownik: prof. dr hab. n. med. Bolesław Samoliński
Adres do korespondencji: Oddział Chorób Dziecięcych i Noworodkowych, Centralny Szpital Kliniczny MSW w Warszawie, ul. Wołoska 137, 02-507 Warszawa, tel.: 22 508 12 20, e-mail: adam.sybilski@cskmswia.pl
Praca finansowana ze środków własnych
Pediatr Med rodz Vol 8 Numer 3, p. 214-221
Our article describes history, clinical features, systemic complications, current practice, and management of Henoch-Schönlein purpura (HSP). HSP is an acute, systemic, small-vessel vasculitis usually seen in healthy children and adults. HSP is the most common vasculitis affecting children. The classic symptoms are: nonthrombocytopenic purpuric rash, arthralgia or arthritis, glomerulonephritis and gastrointestinal involvement: abdominal pain, ulcerations in the stomach, duodenum and colon. More rarely, patients present neurological manifestations: cerebral vasculitis or peripheral nervous system dysfunctions. Although the pathogenetic mechanisms are still not fully described, the role of immunoglobulin A1 (IgA1) in the pathogenesis of the formation of the circulating immune complex was established. Antibody complexes form as a result of infections, drugs, vaccinations and immune mechanisms. There are no specific laboratory tests and markers which can confirm diagnosis. Treatment of HSP is controversial. Recognition of multiorgan manifestations, particularly glomerulonephritis needs the systemic steroids intervention. Angiotensin converting enzyme inhibitor therapy is recommended for reduction of proteinuria. The prognosis depends on severity of renal involvement. Follow-up for children should include: urine testing for proteinuria and haematuria and measurement of a blood pressure for at least 6 months. The risk of complications (proteinuria, hypertension) during pregnancy occurs more often to women with previous history of HSP. Our article describes history, clinical features, systemic complications, current practice, and management.
Keywords: Henoch-Schönlein purpura, diagnosis, vasculitis, nephropathy, purpura