Journal of Nephrology

, Volume 29, Issue 2, pp 203–209 | Cite as

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
Original Article

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.

Keywords

C3 glomerulopathy C3 glomerulonephritis Autoimmune disease ANA ds-DNA 

Notes

Conflict of interest

None.

Ethical approval

This study does not contain any studies with human participants performed by any of the participants.

Informed consent

For this type of study formal consent is not required. The Institutional Review Board at the Mayo Clinic, Rochester, approved the study.

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Copyright information

© Italian Society of Nephrology 2015

Authors and Affiliations

  • Mariam P. Alexander
    • 1
  • Fernando C. Fervenza
    • 2
  • An S. De Vriese
    • 3
  • Richard J. H. Smith
    • 4
  • Samih H. Nasr
    • 1
  • Lynn D. Cornell
    • 1
  • Loren P. Herrera Hernandez
    • 1
  • Yuzhou Zhang
    • 4
  • Sanjeev Sethi
    • 1
  1. 1.Division of Anatomic Pathology, Department of Laboratory Medicine and PathologyMayo ClinicRochesterUSA
  2. 2.Division of Nephrology and Hypertension, Department of Internal MedicineMayo ClinicRochesterUSA
  3. 3.Division of NephrologyAZ Sint-Jan BruggeBruggeBelgium
  4. 4.Otolaryngology and Renal Research Laboratories, Division of Nephrology, Departments of Internal Medicine and PediatricsCarver College of MedicineIowaUSA

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