Pauci-immune Crescentic Glomerulonephritis Masquerading as Goodpasture Syndrome: Ocular Clues in a Young Adult

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Abstract

Introduction: Pauci-immune crescentic glomerulonephritis (PICGN) is the most common cause of rapidly progressive glomerulonephritis, usually associated with antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis. While renal and pulmonary manifestations are well recognized, ocular involvement such as hypertensive retinopathy and exudative retinal detachment is rare. Case Presentation: A 21-year-old previously healthy male developed blurred vision two weeks after a viral illness and NSAID injection. He subsequently presented with malignant hypertension and renal failure requiring dialysis. Ophthalmic evaluation revealed bilateral hypertensive retinopathy with retinal hemorrhages, exudative retinal detachment, and retinoschisis on OCT. Renal biopsy demonstrated 46 glomeruli, of which 14 had cellular crescents and 23 were globally sclerosed. Immunofluorescence was negative for immune complex deposition, confirming pauci-immune crescentic glomerulonephritis. ANCA testing was pending at the time of diagnosis. Corticosteroid therapy was initiated, with plans for additional immunosuppression. Discussion: This case highlights an unusual presentation of ANCA-associated vasculitis with severe ocular involvement. The differential diagnosis included Goodpasture syndrome, lupus choroidopathy, and Vogt–Koyanagi–Harada disease. Renal biopsy was essential for excluding anti-GBM disease and guiding treatment. Multidisciplinary recognition of overlapping retinal and renal pathology is crucial for timely diagnosis and management. Conclusion: Severe ocular findings such as hypertensive retinopathy and exudative retinal detachment may be the first sign of systemic vasculitis. PICGN should be considered in young patients presenting with renal failure and unexplained visual loss. Early renal biopsy and ophthalmic evaluation are critical to prevent irreversible damage.

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