Clinical findings, pathology, and outcomes of C3GN after kidney transplantation

Ladan Zand; Elizabeth C Lorenz; Fernando G Cosio; Fernando C Fervenza; Samih H Nasr; Manish J Gandhi; Richard J H Smith; Sanjeev Sethi

doi:10.1681/ASN.2013070715

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Clinical findings, pathology, and outcomes of C3GN after kidney transplantation

Journal article

Open access

Clinical findings, pathology, and outcomes of C3GN after kidney transplantation

Ladan Zand, Elizabeth C Lorenz, Fernando G Cosio, Fernando C Fervenza, Samih H Nasr, Manish J Gandhi, Richard J H Smith and Sanjeev Sethi

Journal of the American Society of Nephrology, Vol.25(5), pp.1110-1117

05/2014

DOI: 10.1681/ASN.2013070715

PMCID: PMC4005307

PMID: 24357668

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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.

Glomerulonephritis, Membranoproliferative - pathology

Kidney - pathology

Complement C3 - metabolism

Humans

Middle Aged

Male

Treatment Outcome

Glomerulonephritis, Membranoproliferative - surgery

Kidney Transplantation

Paraproteinemias - complications

HLA Antigens - genetics

Young Adult

Glomerulonephritis, Membranoproliferative - immunology

Biopsy

Adolescent

Adult

Female

Child

Glomerulonephritis, Membranoproliferative - metabolism

Details

Title: Subtitle: Clinical findings, pathology, and outcomes of C3GN after kidney transplantation
Creators: Ladan Zand - Department of Internal Medicine, Division of Nephrology and Hypertension, and
Elizabeth C Lorenz - Department of Internal Medicine, Division of Nephrology and Hypertension, and
Fernando G Cosio - Department of Internal Medicine, Division of Nephrology and Hypertension, and
Fernando C Fervenza - Department of Internal Medicine, Division of Nephrology and Hypertension, and
Samih H Nasr - Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota; and
Manish J Gandhi - Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota; and
Richard J H Smith - Otolaryngology and Renal Research Laboratories, Departments of Internal Medicine and Pediatrics, Division of Nephrology, Carver College of Medicine, Iowa City, Iowa
Sanjeev Sethi - Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota; and Sethi.sanjeev@mayo.edu
Resource Type: Journal article
Publication Details: Journal of the American Society of Nephrology, Vol.25(5), pp.1110-1117
DOI: 10.1681/ASN.2013070715
PMID: 24357668
PMCID: PMC4005307
NLM abbreviation: J Am Soc Nephrol
ISSN: 1046-6673
eISSN: 1533-3450
Publisher: United States
Language: English
Date published: 05/2014
Academic Unit: Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Otolaryngology; Internal Medicine
Record Identifier: 9984006485002771

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