Relapsing polychondritis with segmental necrotizing glomerulonephritis.
Glomerulonephritis is a very unusual feature of relapsing polychondritis. A 16-year-old woman presented with microscopic hematuria and severe renal insufficiency during the course of relapsing polychondritis. A renal biopsy specimen disclosed focal segmental proliferative glomerulonephritis with necrotizing lesions and crescent formation. Immunofluorescent studies suggested immune complex mediated glomerular injury. The renal involvement appeared during high-dose steroid therapy. Subsequently, she was treated by adding plasmapheresis and cyclophosphamide during 6 weeks, with sustained improvement. A regimen of plasmapheresis and immunosuppression warrants consideration for use in cases of relapsing polychondritis with glomerulonephritis.
D000293:Adolescent; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005921:Glomerulonephritis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology / Q000628:therapy; D006801:Humans; D007668:Kidney / Q000473:pathology; D010956:Plasmapheresis; D011081:Polychondritis, Relapsing / Q000150:complications* / Q000188:drug therapy / Q000473:pathology / Q000628:therapy; D011241:Prednisone / Q000627:therapeutic use
D003520:Cyclophosphamide; D011241:Prednisone