Granulomatosis with polyangiitis (GPA) / Wengner's granulomatosis : A summary of clinical issues

Abstract

Vasculitis can mimic many diseases; often, there are delays in diagnosis and often ends up in life/organ threatening complications without proper diagnosis and timely treatment. Patients can present with various combinations of constitutional symptoms, vasculitic symptoms, and organ involvements. ANCA tests also have many false positive and false negative results, which adds to the complexity. In addition, Anti-neutrophilic Cytoplasmic Antibody (ANCA) associated Vasculitis (AAV) have different pathological and clinical manifestations from other Vasculitis. There are four types of AAV, including Granulomatosis with Polyangiitis (GPA, previously known as Wegner's Granulomatosis), Microscopic Polyangiitis (MPA), Eosinophilic Granulomatosis with Polyangiitis (E-GPA, previously called as Churg Strauss Vasculitis), and Renal Limited / Organ limited ANCA associated Vasculitis. This review article focuses on the elaboration of the GPA, its differentiation from other Vasculitis, including other ANCAassociated Vasculitis, its complications, and treatment options.