Pauci-immune crescentic glomerulonephritis masquerading as goodpasture syndrome: ocular clues in a young adult: a case report

Pauci-immune crescentic glomerulonephritis (PICGN) is a major cause of rapidly progressive renal failure and is commonly linked to ANCA-associated vasculitis. While renal and pulmonary manifestations are typical, posterior segment ocular involvement—particularly hypertensive retinopathy with exudative retinal detachment and retinoschisis—is rarely described. Reporting such cases is important, as these features may mimic other inflammatory choroidopathies and can delay appropriate diagnosis and treatment. A 21-year-old previously healthy man developed blurred vision shortly after a viral illness and an NSAID injection. He progressed to malignant hypertension and renal failure requiring dialysis. Ophthalmic examination revealed bilateral hypertensive retinopathy with subretinal fluid, pigment epithelial detachments, and retinoschisis on optical coherence tomography. Renal biopsy demonstrated a pauci-immune crescentic glomerulonephritis with 14 cellular crescents and extensive global sclerosis, while immunofluorescence showed no immune complex deposition. Anti-GBM serology was negative, and subsequent testing confirmed p-ANCA (anti-MPO) positivity. The patient was treated with corticosteroids and later initiated on mycophenolate mofetil after exclusion of latent infections. His visual status remained stable on follow-up. This case underscores that severe ocular findings in young adults, particularly when accompanied by malignant hypertension, may signal underlying systemic vasculitis rather than isolated hypertensive damage. Early recognition of atypical retinal features and timely renal biopsy are essential to distinguish PICGN from Goodpasture syndrome and other immune-mediated renal diseases. Awareness of these unusual ocular manifestations may facilitate earlier diagnosis and improve clinical outcomes.

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Publication Details

Journal
BMC Ophthalmology
Published
2025-11-29
DOI
https://doi.org/10.1186/s12886-025-04550-6
Primary Topic
Vasculitis and related conditions
Type
article
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article

Pauci-immune crescentic glomerulonephritis masquerading as goodpasture syndrome: ocular clues in a young adult: a case report

Hathama Razooki Hasan, Aseel Faroun, Batoul Abu Gharbia, Salam Iriqat
BMC Ophthalmology
Vasculitis and related conditions
article

Pauci-immune crescentic glomerulonephritis masquerading as goodpasture syndrome: ocular clues in a young adult: a case report

Hathama Razooki Hasan, Aseel Faroun, Batoul Abu Gharbia, Salam Iriqat
article en

Abstract

Pauci-immune crescentic glomerulonephritis (PICGN) is a major cause of rapidly progressive renal failure and is commonly linked to ANCA-associated vasculitis. While renal and pulmonary manifestations are typical, posterior segment ocular involvement—particularly hypertensive retinopathy with exudative retinal detachment and retinoschisis—is rarely described. Reporting such cases is important, as these features may mimic other inflammatory choroidopathies and can delay appropriate diagnosis and treatment. A 21-year-old previously healthy man developed blurred vision shortly after a viral illness and an NSAID injection. He progressed to malignant hypertension and renal failure requiring dialysis. Ophthalmic examination revealed bilateral hypertensive retinopathy with subretinal fluid, pigment epithelial detachments, and retinoschisis on optical coherence tomography. Renal biopsy demonstrated a pauci-immune crescentic glomerulonephritis with 14 cellular crescents and extensive global sclerosis, while immunofluorescence showed no immune complex deposition. Anti-GBM serology was negative, and subsequent testing confirmed p-ANCA (anti-MPO) positivity. The patient was treated with corticosteroids and later initiated on mycophenolate mofetil after exclusion of latent infections. His visual status remained stable on follow-up. This case underscores that severe ocular findings in young adults, particularly when accompanied by malignant hypertension, may signal underlying systemic vasculitis rather than isolated hypertensive damage. Early recognition of atypical retinal features and timely renal biopsy are essential to distinguish PICGN from Goodpasture syndrome and other immune-mediated renal diseases. Awareness of these unusual ocular manifestations may facilitate earlier diagnosis and improve clinical outcomes.

BMC OphthalmologyVol. 25(1)
An-Najah National University (PS), Al-Quds University (PS), St John of Jerusalem Eye Hospital Group (IL)
Openalex Percentile: Top 44%
Vasculitis and related conditions
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