Late flare-up of nephrotic lupus nephritis transforming histological pattern and autoantibody reactions.
A 15-year-old boy developed a nephrotic syndrome. At that time, autoantibodies related to systemic lupus erythematosus (SLE) had been persistently negative, even on repeated evaluation. C1q was normal, but C4, C3 and CH50 were low. Renal biopsy revealed membranous lupus nephritis (LN) based on the new classification of glomerulonephritis in SLE [Weenig et al. 2004]. We did not establish our diagnosis of SLE on the criteria of the American Rheumatism Association (ARA). The patient showed complete remission ofnephrotic syndrome treated with prednisolone and cyclophosphamide. Thereafter, he had no proteinuria and clinical evidence of SLE for 22 years. At the age of 37, however, he developed facial discoid eruption, proteinuria in the nephrotic range, hypocomplementemia and positive reaction to autoantibodies of SLE. Light microscopic findings of renal biopsy indicated mesangial LN, which showed "full-house" immunofluorescence and mesangial dense deposits associated with diffuse epithelial cell foot process effacement in electron microscopy. Steroid therapy was very effective. This case initially showed autoantibody-negative and hypocomplementemic LN with membranous type, and transformed to SLE with mesangial LN after a long interval.
Tohyama
T
T
Department of Internal Medicine, Kohryo Clinic, Takaoka, Toyama, Japan. ko-cli@p2332.nsk.ne.jp
Ikeda
K
K
Hattori
K
K
D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000032:analysis*; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008181:Lupus Nephritis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone