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

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...

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Main Authors: Boris A. Cruz, Ana Lúcia V. Vaz de Melo, Mariana P. G. Ubirajara Silva, Alberto Kazuo Fuzikawa, Zaida J. Cabrera, Hélcio Antônio Tavares Filho, Felipe Fagioli, Carolina Rutkowski, Abrahão Salomão Filho, Austenir Maciel Coelho, Rejane Pinheiro Damasceno
Format: Article
Language:English
Published: Sociedade Brasileira de Reumatologia
Series:Revista Brasileira de Reumatologia
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Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0482-50042006000600004&lng=en&tlng=en
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author Boris A. Cruz
Ana Lúcia V. Vaz de Melo
Mariana P. G. Ubirajara Silva
Alberto Kazuo Fuzikawa
Zaida J. Cabrera
Hélcio Antônio Tavares Filho
Felipe Fagioli
Carolina Rutkowski
Abrahão Salomão Filho
Austenir Maciel Coelho
Rejane Pinheiro Damasceno
author_facet Boris A. Cruz
Ana Lúcia V. Vaz de Melo
Mariana P. G. Ubirajara Silva
Alberto Kazuo Fuzikawa
Zaida J. Cabrera
Hélcio Antônio Tavares Filho
Felipe Fagioli
Carolina Rutkowski
Abrahão Salomão Filho
Austenir Maciel Coelho
Rejane Pinheiro Damasceno
author_sort Boris A. Cruz
collection DOAJ
description 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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spelling doaj.art-4f71f1d514ef4960b231c62b535660772022-12-21T21:49:48ZengSociedade Brasileira de ReumatologiaRevista Brasileira de Reumatologia1809-4570466380384S0482-50042006000600004Henoch-Schönlein purpura in adults: a case series from a multidisciplinary study groupBoris A. Cruz0Ana Lúcia V. Vaz de Melo1Mariana P. G. Ubirajara Silva2Alberto Kazuo Fuzikawa3Zaida J. Cabrera4Hélcio Antônio Tavares Filho5Felipe Fagioli6Carolina Rutkowski7Abrahão Salomão Filho8Austenir Maciel Coelho9Rejane Pinheiro Damasceno10Biocor InstitutoBiocor InstitutoBiocor InstitutoBiocor InstitutoBiocor InstitutoBiocor InstitutoInstituto Mineiro de NefrologiaInstituto Mineiro de NefrologiaInstituto Mineiro de NefrologiaHospital Governador Israel PinheiroHospital Governador Israel PinheiroBACKGROUND: 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0482-50042006000600004&lng=en&tlng=enpúrpura de Henoch-Schönleinvasculite sistêmicaadultos
spellingShingle Boris A. Cruz
Ana Lúcia V. Vaz de Melo
Mariana P. G. Ubirajara Silva
Alberto Kazuo Fuzikawa
Zaida J. Cabrera
Hélcio Antônio Tavares Filho
Felipe Fagioli
Carolina Rutkowski
Abrahão Salomão Filho
Austenir Maciel Coelho
Rejane Pinheiro Damasceno
Henoch-Schönlein purpura in adults: a case series from a multidisciplinary study group
Revista Brasileira de Reumatologia
púrpura de Henoch-Schönlein
vasculite sistêmica
adultos
title Henoch-Schönlein purpura in adults: a case series from a multidisciplinary study group
title_full Henoch-Schönlein purpura in adults: a case series from a multidisciplinary study group
title_fullStr Henoch-Schönlein purpura in adults: a case series from a multidisciplinary study group
title_full_unstemmed Henoch-Schönlein purpura in adults: a case series from a multidisciplinary study group
title_short Henoch-Schönlein purpura in adults: a case series from a multidisciplinary study group
title_sort henoch schonlein purpura in adults a case series from a multidisciplinary study group
topic púrpura de Henoch-Schönlein
vasculite sistêmica
adultos
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0482-50042006000600004&lng=en&tlng=en
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