Epidemiology and genetics of granulomatosis with polyangiitis

AbstractGranulomatosis with polyangiitis (GPA) previously known as Wegener ’s granulomatosis (WG) is a rare rheumatic disease affecting subjects of all ages. Prevalence and incidence of this systemic disease greatly varies across different ethnic groups. GPA is the commonest form of ANCA-associated vasculitis (AAV) with PR3 positivity among 85–95% of the cases. Scient ific investigations of GPA is warranted because its severity, clinical heterogeneity, fast disease manifestation and end-organ damage. The etiology of GPA is still unknown. Major role of HLA and non-HLA genes with immune functions were identified, however, very limited replication was observed in d ifferent ethnic populations. In the present review, we have discussed the updates on the global epidemiology and contribution ofHLA and major non-HLA genes/loci in GPA. We have also highlighted the cross disease association of GPA associated genes that may help in better disease management and predictive medicine. We proposed that high-resolution HLA typing and development of genetic risk model would help in early disease diagnosis and understanding the prognosis.
Source: Rheumatology International - Category: Rheumatology Source Type: research