C3 Glomerulopathy: Ten Years' Experience at Mayo Clinic

Aishwarya Ravindran; Fernando C Fervenza; Richard J H Smith; An S De Vriese; Sanjeev Sethi

doi:10.1016/j.mayocp.2018.05.019

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C3 Glomerulopathy: Ten Years' Experience at Mayo Clinic

Journal article

Open access

Peer reviewed

C3 Glomerulopathy: Ten Years' Experience at Mayo Clinic

Aishwarya Ravindran, Fernando C Fervenza, Richard J H Smith, An S De Vriese and Sanjeev Sethi

Mayo Clinic proceedings, Vol.93(8), pp.991-1008

08/2018

DOI: 10.1016/j.mayocp.2018.05.019

PMCID: PMC6312642

PMID: 30077216

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https://www.ncbi.nlm.nih.gov/pmc/articles/6312642View

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Abstract

To describe the clinicopathological features, complement abnormalities, triggers, treatment, and outcomes of C3 glomerulopathy. A total of 114 patients with C3 glomerulopathy seen at Mayo Clinic from January 1, 2007, through December 31, 2016, were evaluated in this study. The mean age at diagnosis for the entire cohort was 40.4±22.3 years, with a median serum creatinine level and proteinuria value of 1.6 mg/dL (range: 0.3-14.7) (to convert to mmol/L, multiply by 0.0259) and 2605 mg/24 h (range: 233-24,165), respectively. Hematuria was present in 100 patients (87.7%). The C3 and C4 levels were low in 50 of 112 (44.6%) and 13 of 110 (11.8%) patients, respectively. A history of infection, positive autoimmune findings, and monoclonal gammopathy (MIg) were present in 33 of 114 (28.9%), 28 of 114 (24.6%), and 36 of 95 (37.9%) patients, respectively. However, 28 of 43 patients 50 years or older (65.1%) had MIg. A genetic variant in complement genes, C3 nephritic factor (C3Nef), and other autoantibodies was present in 26 of 70 (37.1%), 30 of 69 (43.5%), and 9 of 67 (13.4%) patients, respectively. Membranoproliferative and mesangial proliferative glomerulonephritis were the common patterns of injury. Patients without MIg were younger (mean age, 32.3±20.6 years), with a median serum creatinine level and proteinuria value of 1.4 mg/dL (range: 0.3-7.9) and 2450 mg/24 h (range: 250-24, 165) and with low C3 and C4 levels in 38 of 77 (49.4%) and 9 of 75 (12.0%) patients, respectively. Most patients received corticosteroids and other immunosuppressive drugs. In patients without MIg, at a median follow-up of 22.3 months (range: 0.1-201.1), the median serum creatinine level and proteinuria value were 1.4 mg/dL (range: 0.3-3.7) and 825.5 mg/24 h (range: 76-22, 603), and 7 patients (9.2%) had progression to end-stage renal disease. C3 glomerulopathy is a heterogeneous disease entity with complex triggering events and abnormalities of the alternative pathway of complement. The disease tends to be progressive and exhibits a variable response to immunosuppressive therapy.

Glucocorticoids - therapeutic use

Hematuria - epidemiology

Autoantibodies - blood

Humans

Immunosuppressive Agents - therapeutic use

Middle Aged

Child, Preschool

Male

Minnesota - epidemiology

Genetic Variation

Young Adult

Paraproteinemias - epidemiology

Immunoglobulins - blood

Aged, 80 and over

Adult

Female

Child

Complement C3 - genetics

Autoimmune Diseases - epidemiology

Infections - epidemiology

Proteinuria - epidemiology

Glomerulonephritis - blood

Glomerulonephritis - genetics

Complement C3 Nephritic Factor - analysis

Disease Progression

Glomerulonephritis - drug therapy

Complement System Proteins

Kidney Failure, Chronic - epidemiology

Adolescent

Creatinine - blood

Nephrotic Syndrome - epidemiology

Aged

Complement Factor H - genetics

Details

Title: Subtitle: C3 Glomerulopathy: Ten Years' Experience at Mayo Clinic
Creators: Aishwarya Ravindran - Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN
Fernando C Fervenza - Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN
Richard J H Smith - Molecular Otolaryngology and Renal Research Laboratories, Division of Nephrology, Department of Internal Medicine, Carver College of Medicine, Iowa City, IA; Department of Pediatrics, Carver College of Medicine, Iowa City, IA
An S De Vriese - Division of Nephrology, Department of Internal Medicine, AZ Sint-Jan Brugge-Oostende, Brugge, and Ghent University, Ghent, Belgium
Sanjeev Sethi - Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN. Electronic address: sethi.sanjeev@mayo.edu
Resource Type: Journal article
Publication Details: Mayo Clinic proceedings, Vol.93(8), pp.991-1008
DOI: 10.1016/j.mayocp.2018.05.019
PMID: 30077216
PMCID: PMC6312642
NLM abbreviation: Mayo Clin Proc
ISSN: 0025-6196
eISSN: 1942-5546
Publisher: England
Grant note: R01 DK110023 / NIDDK NIH HHS
Language: English
Date published: 08/2018
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: 9984006367702771

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