Recurrent primary disease and de novo nephritis following renal transplantation

Abstract

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.

Familial Focal Glomerulosclerosis: A Genetic Linkage to the HLA Locus?

“…The genetic links to focal glomerulosclerosis are high lighted by data derived from the transplant population where the recurrence rate of focal glomerulosclerosis in transplanted kidneys ranges from 35 to 50% [8], In chil dren the rate of recurrence is 25% and half of these pro gress to renal failure [9]. The major risk factor for disease recurrence is age less than 15 years and children under 5 years have the worst prognosis with a recurrence rate in the order of 50%.…”

“…The major risk factor for disease recurrence is age less than 15 years and children under 5 years have the worst prognosis with a recurrence rate in the order of 50%. Recurrence in young children is fre quently associated with renal impairment and progres sion to graft failure within 3 years [9], The recurrence rate of focal glomerulosclerosis in transplants from living related donors and cadaver kidneys is 64 and 35%, respectively [6], On further examination of living related donor transplants, 4 antigen-matched kidneys had an 82% recurrence rate while the recurrence rate for 3 or less antigens fell to 53%, which is not significantly different from cadaveric donor recurrence rates. The high recur rence rate in fully matched siblings suggests that fully matched living related transplants should be avoided in the treatment of focal glomerulosclerosis [6].…”

“…The high recur rence rate in fully matched siblings suggests that fully matched living related transplants should be avoided in the treatment of focal glomerulosclerosis [6]. The associa tion of either haplo identity or living related donor trans plantation was not supported in the paediatric population where age is the greatest risk factor for disease recurrence [9,10]. It remains a distinct possibility that, as living related donor transplantation is most frequently per formed in children [10], earlier reports describing an increased rate of disease recurrence in living related donor transplantation compared to cadaveric transplants were not adequately controlled for age.…”

The clinical spectrum of type IV collagen mutations

“…Alport patients who reach ESRD are dialyzed or undergo renal transplantation. Some transplanted patients develop a posttransplant anti-GBM nephritis, leading to irreversible graft failure (Cameron, 1991).…”

Glomerular disease of transplanted kidneys

“…When transplantation is delayed until no more anti-GMB antibodies are demonstrable, rapidly progressive forms of glomerulonephritis are also rare. 4. Compared with transplant glomerulopathy and recurrent glomerulonephritis, the occurrence 841 of de novo glomerulonephritis in transplants is really rare.…”