C3 glomerulonephritis and autoimmune disease: more than a fortuitous association?

Mariam P Alexander; Fernando C Fervenza; An S De Vriese; Richard J H Smith; Samih H Nasr; Lynn D Cornell; Loren P Herrera Hernandez; Yuzhou Zhang; Sanjeev Sethi

doi:10.1007/s40620-015-0218-9

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C3 glomerulonephritis and autoimmune disease: more than a fortuitous association?

Journal article

Peer reviewed

C3 glomerulonephritis and autoimmune disease: more than a fortuitous association?

Mariam P Alexander, Fernando C Fervenza, An S De Vriese, Richard J H Smith, Samih H Nasr, Lynn D Cornell, Loren P Herrera Hernandez, Yuzhou Zhang and Sanjeev Sethi

Journal of nephrology, Vol.29(2), pp.203-209

04/2016

DOI: 10.1007/s40620-015-0218-9

PMID: 26187133

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Abstract

C3 glomerulonephritis (C3GN) results from genetic or acquired dysregulation of the alternative complement pathway. A subset of patients may have clinical and biochemical characteristics compatible with an autoimmune disorder. We studied a cohort of 85 patients with confirmed C3GN (2007-2014), of which ten patients (3 male, 7 female; mean age 38.5 years) had an associated autoimmune disorder. All patients had abnormal ANA titers, 6 also had positive ds-DNA titers. At the time of presentation with C3GN, all 7 female patients had autoimmune-related presentations. Of the 3 male patients, only 1 patient had autoimmune-related presentations. Kidney biopsy showed predominantly mesangial proliferative or membranoproliferative glomerulonephritis. In 5 patients, the alternative pathway was evaluated. All had allele variants/polymorphisms associated with C3GN. One patient was also positive for C3Nefs. Treatment varied form conservative management to the use of prednisone alone or with cytotoxic therapy. Mean serum creatinine decreased from 2.0 to 1.4 mg/dL while proteinuria decreased from 2300 to 994 mg/24 h in 8 patients with follow-up. The study highlights the association between C3GN and autoimmune disorders, particularly in female patients. The study suggests that an autoimmune milieu may act as a trigger for the development of C3GN in genetically susceptible patients. Short-term prognosis of C3GN associated with autoimmune disorders appears excellent.

Glucocorticoids - therapeutic use

Humans

Middle Aged

Autoimmunity - genetics

Male

Autoimmune Diseases - genetics

Glomerulonephritis, Membranoproliferative - drug therapy

Young Adult

Adult

Female

Drug Therapy, Combination

Complement C3 - genetics

Autoimmune Diseases - drug therapy

Complement Activation - drug effects

Genetic Predisposition to Disease

Kidney Glomerulus - drug effects

Risk Factors

Autoimmune Diseases - immunology

Treatment Outcome

Complement C3 Nephritic Factor - analysis

Autoimmune Diseases - diagnosis

Biomarkers - blood

Genetic Markers

Kidney Glomerulus - pathology

Complement C3 - immunology

Complement Activation - genetics

Glomerulonephritis, Membranoproliferative - genetics

Glomerulonephritis, Membranoproliferative - immunology

Phenotype

Sex Factors

Glomerulonephritis, Membranoproliferative - diagnosis

Kidney Glomerulus - immunology

Autoimmunity - drug effects

Prednisone - therapeutic use

Details

Title: Subtitle: C3 glomerulonephritis and autoimmune disease: more than a fortuitous association?
Creators: Mariam P Alexander - Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, Rochester, MN, 55905, USA
Fernando C Fervenza - Division of Nephrology and Hypertension, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA
An S De Vriese - Division of Nephrology, AZ Sint-Jan Brugge, Brugge, Belgium
Richard J H Smith - Otolaryngology and Renal Research Laboratories, Division of Nephrology, Departments of Internal Medicine and Pediatrics, Carver College of Medicine, Iowa, IA, USA
Samih H Nasr - Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, Rochester, MN, 55905, USA
Lynn D Cornell - Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, Rochester, MN, 55905, USA
Loren P Herrera Hernandez - Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, Rochester, MN, 55905, USA
Yuzhou Zhang - Otolaryngology and Renal Research Laboratories, Division of Nephrology, Departments of Internal Medicine and Pediatrics, Carver College of Medicine, Iowa, IA, USA
Sanjeev Sethi - Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, Rochester, MN, 55905, USA. sethi.sanjeev@mayo.edu
Resource Type: Journal article
Publication Details: Journal of nephrology, Vol.29(2), pp.203-209
DOI: 10.1007/s40620-015-0218-9
PMID: 26187133
NLM abbreviation: J Nephrol
ISSN: 1121-8428
eISSN: 1724-6059
Publisher: Italy
Language: English
Date published: 04/2016
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: 9984006349102771

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