Henoch-Schönlein purpura in adults: a case series from a multidisciplinary study group

Revista Brasileira de Reumatologia

Boris A. Cruz²  Ana Lúcia V. Vaz De Melo²  Mariana P. G. Ubirajara Silva¹  Alberto Kazuo Fuzikawa¹  Zaida J. Cabrera¹  Hélcio Antônio Tavares Filho¹  Felipe Fagioli¹  Carolina Rutkowski¹  Abrahão Salomão Filho¹  Austenir Maciel Coelho¹  Rejane Pinheiro Damasceno¹ 

[1] ,Biocor Instituto Rheumatology Department

关键词: Henoch-Schönlein purpura;    systemic vasculitis;    adults;    púrpura de Henoch-Schönlein;    vasculite sistêmica;    adultos;   

DOI  :  10.1590/S0482-50042006000600004

来源: SciELO

【 摘 要 】

BACKGROUND: Henoch-Schönlein purpura (HSP) is a systemic vasculitis involving small vessels with the deposition of immune complexes containing IgA. It has been extensively studied in children, but in adults, its natural history is much less known. OBJECTIVES: to report a series of patients with HSP presenting in their adulthood. PATIENTS AND METHODS: the Minas Gerais Vasculitis Study Group´s Members (MGVSG) were invited to report patients with HSP who appeared in their adulthood. A standardized retrospective chart review was done. RESULTS: eleven patients, two male and nine female, age 39.4 +/- 20.1 yearsold were studied. Nine patients presented purpura, seven presented arthritis, four patients had gastrointestinal involvement and ten patients (91%) presented glomerulonephritis (GN). Eight patients were subjected to renal biopsies. Six of them presented endocapillary proliferative GN and only two of them had minimal mesangial proliferation. In the other three patients, HSP was confirmed by skin biopsies. All patients received steroids, in five of them steroids were combined with other immunosuppressive agents. After a follow-up of 39.0 +/- 64.6 months, four patients (36%) presented impairment of renal function, but only one (9%) developed end stage renal disease and was successfully appeared to renal transplantation. At the end of follow-up, seven patients (64%) are in complete remission and four in partial remission. CONCLUSION: in adulthood, HSP represents a distinct clinical syndrome with a higher frequency of renal involvement and more severe systemic vasculitis. Nevertheless, the final outcome in this series was as good as reported in children, maybe due to aggressive immunosuppressive therapy.

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