Nephrotic range proteinuria and rapid progression to end-stage kidney disease in seronegative pauci-immune necrotizing glomerulonephritis masked by cutaneous lesions: a case-based review.
Aleksandra Gałan, Filip Koszałka, Izabela Zakrocka et al.
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Case in brief
A 22-year-old woman with a history of recurrent cutaneous lesions was hospitalized due to exacerbation of her skin condition. Laboratory tests revealed nephrotic-range proteinuria and reduced eGFR. Renal biopsy showed cellular crescents and significant inflammatory infiltrates, leading to a diagnosis of ANCA-negative pauci-immune necrotizing glomerulonephritis. Despite treatment with methylprednisolone, her renal function deteriorated, requiring renal replacement therapy.
Diagnostic / clinical pearl
Clinicians should maintain a high index of suspicion for ANCA-negative pauci-immune necrotizing glomerulonephritis in patients with compatible systemic and renal findings, even in the absence of serologic markers.
Why it matters
This case highlights the importance of renal biopsy in diagnosing atypical presentations of glomerular diseases, particularly when serologic tests are negative.