Ciclosporin-Induced Partial and Transient Improvement of Nephrotic Syndrome in Recurrent Focal Segmental Glomerulosclerosis

A 2.8-year-old girl with focal segmental glomerulosclerosis had recurrence of nephrotic syndrome within 3 days of renal transplantation and the serum creatinine increased. Renal biopsy showed cellular rejection and also complete effacement of the epithelial cell foot processes. The rejection responded to methylprednisolone therapy but massive proteinuria persisted. An increase in the dose of cyclosporine A to 14 mg/kg per day was followed by immediate remission of the proteinuria. One month later, a second renal biopsy showed only focal fusion of foot processes. She remains free of proteinuria 2 years later. We propose that the higher dose of cyclosporine caused remission of the nephrotic syndrome.

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Recurrent primary disease and de novo nephritis following renal transplantation

1991

Pediatr Nephrol

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Recurrent or de novo diseases account for only 5% of graft failure in children, but have much to teach us about mechanisms. In children, almost the only metabolic disease with recurrence is type I hyperoxaluria, in which the poor long-term results of isolated renal transplantation make combined liver and renal transplantation, or even prophylactic liver transplantation before renal failure the preferable alternatives. While many forms of nephritis may show histological recurrence in allografts, it is notable that in many patients this is accompanied by no clinical manifestations or only mild disease: this is particularly so in mesangiocapillary glomerulonephritis (MCGN) type II. IgA-associated nephropathy and Henoch-Schönlein purpura. However focal segmental glomerulosclerosis and MCGN type I recur with sufficient frequency and severity to deter the use of living donors unless there is no alternative. The same is true of haemolytic-uraemic syndromes. As many as 10% of paediatric grafts may show de novo membranous nephropathy, but in the majority this is mild or not clinically evident. In contrast, the rare anti-glomerular basement membrane nephritis affecting some patients with Alport's syndrome usually results in graft failure, but occurs in only a minority of recipients with the syndrome. For all types of disease in allografts, risk factors for recurrence are poorly worked out, and attempts at treatment generally ineffective.

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Ciclosporin-Induced Partial and Transient Improvement of Nephrotic Syndrome in Recurrent Focal Segmental Glomerulosclerosis

Cited by 9 publications

References 4 publications

Recurrence of glomerulonephritis following renal transplantation

Recurrence of glomerulonephritis following renal transplantation

Prompt remission of post-renal transplant nephrotic syndrome with high-dose cyclosporine

Recurrent primary disease and de novo nephritis following renal transplantation

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