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NDT Plus Advance Access originally published online on October 31, 2008
NDT Plus 2009 2(1):34-35; doi:10.1093/ndtplus/sfn165
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© The Author [2008]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

Familial clusters of ANCA small-vessel vasculitis

Ana Marta Gomes1, Filipe Nery2, Ana Ventura1, Clara Almeida1 and Joaquim Seabra1

1 Department of Nephrology, Centro Hospitalar Vila Nova Gaia/Espinho, Gaia
2 Department of Internal Medicine, Centro Hospitalar do Porto-Hospital Sto António, Porto, Portugal

Correspondence: Correspondence and offprint requests to: Ana Marta Gomes, Department of Nephrology, Centro Hospitalar Vila Nova Gaia/Espinho, Rua Conceição Fernandes, 3343-502 Vila Nova de Gaia, Portugal. Tel: +351919859507; Fax: +351227538982; E-mail: ampgomes{at}gmail.com


  Abstract

Small-vessel vasculitides associated with the presence of antineutrophil cytoplasmic antibodies in the serum are characterized by inflammation and necrosis of small vessels. A pauci-immune necrotizing crescentic glomerulonephritis typically occurs when there is renal damage. Pathogenesis of these diseases remains unclear although infectious, genetic and environmental factors have been involved. Few familial clusters of antineutrophil cytoplasmic antibodies’ small-vessel vasculitis are described in the literature. We report two families with first-degree relatives affected with antineutrophil cytoplasmic antibodies’ small-vessel vasculitis.

Key Words: antineutrophil cytoplasmatic antibodies • familial vasculitis • systemic vasculitis

Received for publication August 8, 2008. Accepted for publication October 6, 2008.


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