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QUIZ No. 1
CASE 1
A young patient with end-stage renal disease on chronic hemodialysis presents with acute onset chest pain and hemoptysis. CT thorax done (suboptimal due to poor patient co-peration). What is the most likely diagnosis?
CT
Diagnosis – Pulmonary renal syndrome – Granulomatosis with polyangiitis
Findings - Multiple bilateral peripheral pulmonary nodules, some showing cavitation. Associated patchy ground-glass opacities, suggestive of alveolar haemorrhage. Bilateral moderate pleural effusions. Gross free fluid in abdomen. No focal lobar consolidation or tree-in-bud pattern to strongly suggest infection
Discussion
Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis predominantly affecting small- to medium-sized vessels, with a characteristic predilection for the upper respiratory tract, lungs, and kidneys.
Immune-mediated necrotizing granulomatous vasculitis with pauci-immune crescentic glomerulonephritis; classically c-ANCA (PR3) positive.
CT shows multiple nodules or consolidations with cavitation, peripheral or random distribution, alveolar hemorrhage, and possible pleural effusion.
Treatment - High-dose corticosteroids with cyclophosphamide or rituximab; plasma exchange in severe pulmonary hemorrhage.
Teaching Pearl:
Young patient + Dialysis + hemoptysis + cavitating peripheral lung lesions = think vasculitis (GPA) before infection.
P.S - The diagnosis was confirmed by c-ANCA positivity.
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