Abstract

Granulomatosis with polyangiitis (GPA) is a potentially lethal systemic disorder that is characterized by necrotizing vasculitis of small arteries and veins. The respiratory system is most commonly affected in limited forms of the disease, however upper and lower respiratory system, systemic vasculitis, and necrotizing glomerulonephritis are the characteristic components of the disease triad. We present a case in a young male adult patient presenting with symptoms of sinusitis, joint pains, fever with detection of lung lesions in initial chest x ray and CT scan and liver involvement on further investigations and pathological confirmation by antineutrophil cytoplasmic antibody positivity and nasal ulcers. After treatment with corticosteroid and IV Rituximab, the lung findings were entirely resolved with improvement in liver functions.

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