Abstract

The recurrence of glomerulonephritis (GN) is critical to the prognosis of long-term renal transplant graft survival. C3 GN is a rare GN with a poor prognosis, and there are only a few reports of recurrent cases after renal transplantation. We recently experienced a case highly suspected of C3 GN recurrence. The patient was a 67-year-old male who had suffered from end-stage renal disease (ESRD) and started dialysis at the age of 60. His primary renal disease was unknown. At the age of 63, renal transplantation was successfully performed. His serum creatinine (Cr) level was maintained at 1.2 to 1.5 mg/dl, with no urinary protein or occult blood until 18 months after the transplant when urinary occult blood and protein became constant, along with an elevated Cr level at 1.5 to 1.6 mg/dl. The tentative diagnosis following a protocol biopsy at 36 months was dense-deposit disease. However, the pathological findings of an episode biopsy performed 1 year later, when his Cr level elevated to 2.0 mg/dl after varicella zoster virus reactivation, revealed no acute rejection but were compatible with C3 GN. These findings are consistent with a previous report on recurrent C3 GN that revealed relatively rapid loss of graft function, contrasting well with slow progression occurring in the native kidney. This case is possibly the first report in Japan of recurrent C3 GN after renal transplantation.

Highlights

  • The recurrence of glomerulonephritis (GN) is critical to the prognosis of long-term renal transplant graft survival

  • These findings are consistent with a previous report on recurrent C3 GN that revealed relatively rapid loss of graft function, contrasting well with slow progression occurring in the native kidney

  • C3 glomerulopathy (C3GP) is a rare disease that is due to abnormalities in the alternative pathway (AP) suspected of being caused either by autoantibodies or genetic abnormalities in the AP pathway

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Summary

Conclusions

Compared to the native kidney, early recurrence and rapid loss of graft function was observed in our case. No recurrence at 2 months after transplantation (C3NeF +). Recurrence at 1 month after transplantation with proteinuria and normal renal function (C3NeF −). Kidney function is stable 9 years after transplantation. Recurrence of GN with isolated C3 deposits 5 months later after kidney transplantation and TMA 4 years later. Abbreviations AP, alternative pathway; C3GP, C3 glomerulopathy; C3NeF, C3 nephritic factor; CFH, complement factor H; CFHR1-5, complement factor H-related 1-5; CHI, complement factor I; Cr, creatinine; DDD, dense-deposit disease; EM, electron microscopy; ESRD, end-stage renal disease; GN, glomerulonephritis; IF, immunofluorescence; Ig, immunogloblulin; LM, light microscopy; MMF, mycophenolate mofetil; MPGN, membranoproliferative glomerulonephritis; POY, post-operative year; PSL, prednisolone; TAC, tacrolimus; TMA, thrombotic microangiopathy; VZV, varicella zoster virus. Author details 1Department of Nephrology and Dialysis, Tazuke Kofukai Medical Research Institute, Kitano Hospital, 2-4-20 Ohgimachi, Kita-ku, Osaka 530-8480, Japan. Author details 1Department of Nephrology and Dialysis, Tazuke Kofukai Medical Research Institute, Kitano Hospital, 2-4-20 Ohgimachi, Kita-ku, Osaka 530-8480, Japan. 2Department of Nephrology, Kansai Electric Power Hospital, 2-1-7 Fukushima, Fukusima-ku, Osaka 553-0003, Japan. 3Department of Nephrology, Kyoto University Hospital, 54 Shogoin-Kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan

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