Abstract
Granulomatosis with angitis, formerly termed Wegener’s granulomatosis (WG) is an uncommon autoimmune disease with multi‑system involvement, the pathological hallmark of which is necrotizing granulomas with vasculitis. Although it commonly limits itself to involvement of the upper and lower respiratory tracts or kidneys, it may potentially involve any organ. We present the case of a young lady who presented with persistent fever lasting 2 months and right upper lobe consolidation, with laboratory evidence of glomerulonephritis. Serological tests revealed high titres of antibody to proteinase 3 (antineutrophil cytoplasmic antibody – cytoplasmic type- ANCA). A lung biopsy from the left upper lobe revealed necrotizing granulomas with vasculitis, thus clinching the diagnosis. Remission was achieved with a combination of prednisolone with rituximab, complete remission being evidenced as early as 3 weeks of therapy initiation. Steroids were gradually tapered in the maintenance phase and she remains under close follow up for any features of relapse.
Keywords: Granulomatosis with angitis, necrotizing granulomas, small vessel vasculitis, Wegener’s granulomatosis
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Corresponding Author
Dr Rajesh V
Consultant Pulmonologist, Department of Pulmonary Medicine, Rajagiri Hospital, Aluva, Kerala, 683112
Email: This email address is being protected from spambots. You need JavaScript enabled to view it., Mobile number: 9446728953, 0484- 29055039
DOI: https://dx.doi.org/10.18535/jmscr/v6i12.84
