title: Clinical findings, pathology, and outcomes of C3GN after kidney transplantation.
abstract: C3 glomerulonephritis (C3GN) results from abnormalities in the alternative pathway of complement, and it is characterized by deposition of C3 with absent or scant Ig deposition. In many patients, C3GN progresses to ESRD. The clinical features, pathology, and outcomes of patients with C3GN receiving kidney transplantation are unknown. Between 1996 and 2010, we identified 21 patients at our institution who received a kidney transplant because of ESRD from C3GN. The median age at the time of initial diagnosis of C3GN at kidney biopsy was 20.8 years. The median time from native kidney biopsy to dialysis or transplantation was 42.3 months. Of 21 patients, 14 (66.7%) patients developed recurrent C3GN in the allograft. The median time to recurrence of disease was 28 months. Graft failure occurred in 50% of patients with recurrent C3GN, with a median time of 77 months to graft failure post-transplantation. The remaining 50% of patients had functioning grafts, with a median follow-up of 73.9 months. The majority of patients had hematuria and proteinuria at time of recurrence. Three (21%) patients were positive for monoclonal gammopathy and had a faster rate of recurrence and graft loss. Kidney biopsy at the time of recurrence showed mesangial proliferative GN in eight patients and membranoproliferative GN in six patients. All allograft kidney biopsies showed bright C3 staining (2-3+), with six biopsies also showing trace/1+ staining for IgM and/or IgG. To summarize, C3GN recurs in 66.7% of patients, and one half of the patients experience graft failure caused by recurrence.
authors.affiliation: Department of Internal Medicine, Division of Nephrology and Hypertension, and.
authors.forename: Ladan
authors.initials: L
authors.lastname: Zand
authors.affiliation: Department of Internal Medicine, Division of Nephrology and Hypertension, and.
authors.forename: Elizabeth C
authors.initials: EC
authors.lastname: Lorenz
authors.affiliation: Department of Internal Medicine, Division of Nephrology and Hypertension, and.
authors.forename: Fernando G
authors.initials: FG
authors.lastname: Cosio
authors.affiliation: Department of Internal Medicine, Division of Nephrology and Hypertension, and.
authors.forename: Fernando C
authors.initials: FC
authors.lastname: Fervenza
authors.affiliation: Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota; and.
authors.forename: Samih H
authors.initials: SH
authors.lastname: Nasr
authors.affiliation: Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota; and.
authors.forename: Manish J
authors.initials: MJ
authors.lastname: Gandhi
authors.affiliation: Otolaryngology and Renal Research Laboratories, Departments of Internal Medicine and Pediatrics, Division of Nephrology, Carver College of Medicine, Iowa City, Iowa.
authors.forename: Richard J H
authors.initials: RJ
authors.lastname: Smith
authors.affiliation: Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota; and Sethi.sanjeev@mayo.edu.
authors.forename: Sanjeev
authors.initials: S
authors.lastname: Sethi
mesh_terms: D000293:Adolescent; D000328:Adult; D001706:Biopsy; D002648:Child; D003176:Complement C3 / Q000378:metabolism*; D005260:Female; D015432:Glomerulonephritis, Membranoproliferative / Q000276:immunology* / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D006680:HLA Antigens / Q000235:genetics; D006801:Humans; D007668:Kidney / Q000473:pathology; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D003176:Complement C3; D006680:HLA Antigens