Clinical and histological improvement with long-term, low-dose cyclosporin A in a patient with severe IgA nephropathy

We report the case of an 11-year-old girl with nephrotic syndrome with massive proteinuria and microscopic hematuria. Her first renal biopsy specimen (June 1997) showed diffuse/segmental mesangial proliferative glomerulonephritis with capillary wall thickening, crescent, and sclerosis by light microscopy, as well as diffuse/global moderate deposition of IgA, C3, and fibrinogen predominantly in the mesangium, and partly along the capillary wall, by immunofluorescent microscopy. After the patient failed to show remission with the usual dose of prednisolone and azathioprine, cyclosporin A was administered, in addition to dipyridamole, warfarin, and prednisolone (on alternative days). In consequence, the proteinuria had completely disappeared after 6 weeks of this regimen and microscopic hematuria had disappeared after 8 months of the regimen. A second renal biopsy was performed in August 1998. The epithelial proliferation and crescent seen in the first biopsy specimen had disappeared, and only mesangial proliferation and sclerosis persisted, without histological evidence of cyclosporin-induced nephrotoxicity. A third renal biopsy was performed in March, 2000. IgA deposition in glomeruli had disappeared in this biopsy specimen. Low-dose cyclosporin A therapy resulted in dramatic improvements in both clinical manifestations and renal histological findings, without detrimental effects on renal function.