Familial vasculitides: granulomatosis with polyangitis and microscopic polyangitis in two brothers with differing anti-neutrophil cytoplasm antibody specificity
File(s)
Published version
Author(s)
Prendecki, M
Cairns, T
Pusey, CD
Type
Journal Article
Abstract
Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a group of rare autoimmune diseases. Although the aetiology of AAV is uncertain, it is likely that genetic and environmental factors contribute. We report the unusual case of two brothers presenting with AAV with differing clinical pictures and differing ANCA specificity. There is a recently identified difference in genetic risk factors associated with ANCA specificity, making it surprising that first-degree relatives develop AAV with differing clinical and serological features. Our report illustrates the complex aetiology of AAV and suggests that further research on the interaction of genetic and environmental factors is needed.
Date Issued
2016-04-14
Date Acceptance
2016-02-26
Citation
Clinical Kidney Journal, 2016, 9 (3), pp.429-431
ISSN
2048-8513
Publisher
Oxford University Press
Start Page
429
End Page
431
Journal / Book Title
Clinical Kidney Journal
Volume
9
Issue
3
Copyright Statement
© The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited.
License URL
Sponsor
Imperial College Healthcare NHS Trust- BRC Funding
Identifier
PII: sfw016
Grant Number
RDA04 79560
Subjects
ANCA
familial
vasculitis
Publication Status
Published