A Case of Granulomatosis with Polyangiitis (Wegener’s Granulomatosis) Presenting with Rapidly Progressive Glomerulonephritis

Abstract

Granulomatosis with polyangiitis (GPA, Wegener’s granulomatosis) presenting as rapidly progressive glomerulonephritis is not uncommon. The recognition of multisystem disease involving joints, kidney, and lung is critical for diagnosing Wegener's vasculitis. Here, we report a case study of a 52-year-old Bangladeshi man presented with a history of progressively worsening fever, recurrent cough, and hemoptysis. He developed renal failure within a month which was successfully treated with high-dose steroids, cyclophosphamide, and trimethoprim-sulfamethoxazole (TMP-SMX). Rapidly progressive glomerulonephritis can be a fulminant manifestation of GPA, in which case an immediate and aggressive treatment with pulse steroids, high-dose cyclophosphamide and TMP-SMX can be lifesaving.

Granulomatosis with Polyangiitis Presenting as Refractory Chronic Cough, Manifesting by Massive Alveolar Haemorrhage – A Rare Case Report

“…A combination of enzyme-linked immunosorbent assay and immunofluorescence is reported to have 98.5% specificity and 96% sensitivity for diagnosis of ANCA-associated vasculitis. 9 …”

Granulomatosis With Polyangiitis: A Pauci-Immune Rapidly Progressive Glomerulonephritis With Isolated Renal Involvement in an Elderly Male

“…Granulomatosis with polyangiitis (GPA), also known as Wegener’s granulomatosis, is an antineutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis. It is a multisystem disorder affecting the upper and lower respiratory tracts, and the renal system [ 1 ]. It is characterized by necrotizing vasculitis of the respiratory tract and often involves the renal system late in the course of the disease with focal or segmental glomerulonephritis [ 2 ].…”