ArticleBMJ case reports2012
Familial granulomatosis with polyangiitis: three cases of this rare disorder in one Indoasian family carrying an identical HLA DPB1 allele.
Article in BMJ case reports, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed, 15 citations in OpenAlex.
- Immunopathology of Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis.International journal of molecular sciences · 2025Review
- Association between ATG16L1 rs2241880(T300A) and rs4663421 and ANCA‑associated vasculitis in the Guangxi population of China: Propensity score matching analysis.Biomedical reports · 2025Article
- Familial Association of Granulomatosis With Polyangiitis: A Case-Based Review of Literature.Cureus · 2023Article
- Rituximab used for simultaneous treatment of PR3-ANCA positive vasculitis associated with rheumatoid arthritis: A case report.Clinical case reports · 2020Article
- Familial vasculitides: granulomatosis with polyangitis and microscopic polyangitis in two brothers with differing anti-neutrophil cytoplasm antibody specificity.Clinical kidney journal · 2016Article
- The immunopathology of ANCA-associated vasculitis.Seminars in immunopathology · 2014Review
- Genetic Susceptibility to ANCA-Associated Vasculitis: State of the Art.Frontiers in immunology · 2014Review
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
Abstract
The aetiology of granulomatosis with polyangiitis (GPA) remains unclear. There is likely a complex interplay between environmental triggers and genetic predisposition. Early diagnosis and treatment improve outcome in this condition with an untreated reported mortality of 90%. There are a few cases of familial GPA in the literature, but we report here the interesting and rare scenario of three members of the same Indoasian family with GPA who share an identical human leucocyte antigen (HLA) haplotype. In particular, all three members of the family share the HLADPB1 allele *04 : 01. This is the first reported Indoasian family with GPA. The HLA antigens found on genotyping our family and, in particular, HLADPB1 has been linked with GPA in previous studies. Genome-wide association studies in Anti Neutrophil Cytoplasm Antibody (ANCA) associated vasculitis (AAV) are currently in progress and should improve understanding of the genetic aspects of this disease. There may be important implications for patients with GPA and for their relatives.
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