RESUMO
BACKGROUND/AIM: The aim of this retrospective study was to evaluate the presentation, clinical and pathological manifestations and outcome of the Henoch-Schönlein purpura (HSP) nephritis in children. METHODS: Clinical and laboratory data of 443 children with HSP nephritis aged between 3 and 16 years from 16 pediatric nephrology reference centers were analyzed retrospectively. The biopsy findings were graded according to the classification developed by the International Study of Kidney Disease in Children (ISKDC). RESULTS: Renal biopsy was performed in 179 of the patients with HSP nephritis. The most common presenting clinical finding in patients who were biopsied was nephrotic range proteinuria (25%) which was followed by nephritic-nephrotic syndrome (23.5%). The biopsy findings according to the ISKDC were as follows: class I: 8.3%; II: 44.1%; III: 36.3%; IV: 6.7%; V: 3.3%; VI: 1.1%. All of the patients who developed end-stage renal disease had nephritic-nephrotic syndrome at presentation. Of 443 patients, 87.2% had a favorable outcome and 12.8% had an unfavorable outcome. The overall percentage of children who developed end-stage renal disease at follow-up was 1.1%. Logistic regression analysis did not show any association of initial symptoms and histology with outcome. CONCLUSION: In the presented cohort, the presence of crescents in the first biopsy or presenting clinical findings did not seem to predict the outcome of HSP nephritis in children. We conclude that children with HSP nephritis even with isolated microscopic hematuria and/or mild proteinuria should be followed closely.
Assuntos
Vasculite por IgA/epidemiologia , Vasculite por IgA/patologia , Nefrite/epidemiologia , Nefrite/patologia , Adolescente , Criança , Pré-Escolar , Comorbidade , Feminino , Humanos , Incidência , Masculino , Reprodutibilidade dos Testes , Medição de Risco , Fatores de Risco , Sensibilidade e Especificidade , Turquia/epidemiologiaRESUMO
The cutaneous form of polyarteritis nodosa in children is extremely rare. Findings are usually limited to the skin, muscles and joints. It has a benign but often chronic course. We describe an 8-y-old girl with cutaneous PAN, with extensive livedo reticularis on lower and upper extremities, tender subcutaneous nodules, arthralgia and right ankle swelling. Skin biopsy revealed vasculitis of small and medium-sized blood vessels characterized by fibrinoid necrosis. The use of prednisolone resulted in clinical improvement initially, but recurrence occurred during tapering. She showed marked improvement with additional high dose methyl prednisolone monthly.
