Skip to main content

Advertisement

SpringerLink
Log in

Membranoproliferative glomerulonephritis associated with autoimmune diseases

  • Original Article
  • Published:
Journal of Nephrology Aims and scope Submit manuscript

Abstract

Membranoproliferative glomerulonephritis (MPGN) has been classified based on its pathogenesis into immune complex-mediated and complement-mediated MPGN. The immune complex-mediated type is secondary to chronic infections, autoimmune diseases or monoclonal gammopathy. There is a paucity of data on MPGN associated with autoimmune diseases. We reviewed the Mayo Clinic database over a 10-year period and identified 12 patients with MPGN associated with autoimmune diseases, after exclusion of systemic lupus erythematosus. The autoimmune diseases included rheumatoid arthritis, primary Sjögren’s syndrome, undifferentiated connective tissue disease, primary sclerosing cholangitis and Graves’ disease. Nine of the 12 patients were female, and the mean age was 57.9 years. C4 levels were decreased in nine of 12 patients tested. The serum creatinine at time of renal biopsy was 2.2 ± 1.0 mg/dl and the urinary protein was 2,850 ± 3,543 mg/24 h. Three patients required dialysis at the time of renal biopsy. Renal biopsy showed an MPGN in all cases, with features of cryoglobulins in six cases; immunoglobulin (Ig)M was the dominant Ig, and both subendothelial and mesangial electron dense deposits were noted. Median follow-up was 10.9 months. Serum creatinine and proteinuria improved to 1.6 ± 0.8 mg/dl and 428 ± 677 mg/24 h, respectively, except in 3 patients with end-stage renal disease. In summary, this study describes the clinical features, renal biopsy findings, laboratory evaluation, treatment and prognosis of MPGN associated with autoimmune diseases.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Sethi S (2011) Fervenza FC: membranoproliferative glomerulonephritis: pathogenetic heterogeneity and proposal for a new classification. Semin Nephrol 31:341–348

    Article  CAS  PubMed  Google Scholar 

  2. Sethi S, Fervenza FC (2012) Membranoproliferative glomerulonephritis: a new look at an old entity. N Engl J Med 366:1119–1131

    Article  CAS  PubMed  Google Scholar 

  3. Servais A, Fremeaux-Bacchi V, Lequintrec M, Salomon R, Blouin J, Knebelmann B, Grunfeld J-P, Lesavre P, Noel L, Fakhouri F (2007) Primary glomerulonephritis with isolated c3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome. J Med Genet 44:193–199

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  4. Sethi S, Fervenza FC, Zhang Y, Nasr SH, Leung N, Vrana J, Cramer C, Nester CM, Smith RJH (2011) Proliferative glomerulonephritis secondary to dysfunction of the alternative pathway of complement. Clin J Am Soc Nephrol 6:1009–1017

    Article  PubMed Central  PubMed  Google Scholar 

  5. Sethi S, Fervenza FC, Zhang Y, Zand L, Vrana JA, Nasr SH, Theis JD, Dogan A, Smith RJH (2012) C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up. Kidney Int 82:465–473

    Article  CAS  PubMed  Google Scholar 

  6. Rennke H (1995) Secondary membranoproliferative glomerulonephritis. Kidney Int 47:643–656

    Article  CAS  PubMed  Google Scholar 

  7. Goules A, Masouridi S, Tzioufas AG, Ioannidis JPA, Skopouli FN, Moutsopoulos HM (2000) Clinically significant and biopsy-documented renal involvement in primary Sjögren syndrome. Medicine 79:241–249

    Article  CAS  PubMed  Google Scholar 

  8. Cortez MS, Sturgill BC, Bolton WK (1995) Membranoproliferative glomerulonephritis with primary Sjögren’s syndrome. Am J Kidney Dis 25:632–636

    Article  CAS  PubMed  Google Scholar 

  9. Ting H, Wang F (1977) Mesangiocapillary (membranoproliferative) glomerulonephritis and rheumatoid arthritis. Br Med J 1:270–271

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  10. Helin H, Korpela M, Mustonen J, Pasternack A (1995) Renal biopsy findings and clinicopathologic correlations in rheumatoid arthritis. Arthritis Rheum 38:242–247

    Article  CAS  PubMed  Google Scholar 

  11. Gurkan S, Dikman S, Saland MJ (2009) A case of autoimmune thyroiditis and membranoproliferative glomerulonephritis. Pediatr Nephrol 24:193–197

    Article  PubMed  Google Scholar 

  12. Jhaveri KD, D’Agati VD, Pursell R, Serur D (2009) Coeliac sprue-associated membranoproliferative glomerulonephritis (MPGN). Nephrol Dial Transpl 24:3545–3548

    Article  CAS  Google Scholar 

  13. Gakiopoulou H, Korkolopoulou P, Paraskevakou H, Marinaki S, Voulgarelis M, Stofas A, Lelouda M, Lazaris AC, Boletis J, Patsouris E (2010) Membranoproliferative glomerulonephritis in the setting of multicentric angiofollicular lymph node hyperplasia (castleman’s disease) complicated by Evan’s syndrome. J Clin Pathol 63:552–554

    Article  PubMed  Google Scholar 

  14. Weening JJ, D’Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F, Fogo AB, Ginzler EM, Hebert L, Hill G, Hill P, Jennette JC, Kong NC, Lesavre P, Lockshin M, Looi L-M, Makino H, Moura LA, Nagata M (2004) The classification of glomerulonephritis in systemic lupus erythematosus revisited. J Am Soc Nephrol 15:241–250

    Article  PubMed  Google Scholar 

  15. Neogi T, Aletaha D, Silman AJ, Naden RL, Felson DT, Aggarwal R, Bingham CO, Birnbaum NS, Burmester GR, Bykerk VP, Cohen MD, Combe B, Costenbader KH, Dougados M, Emery P, Ferraccioli G, Hazes JMW, Hobbs K, Huizinga TWJ, Kavanaugh A, Kay J, Khanna D, Kvien TK, Laing T, Liao K, Mease P, Ménard HA, Moreland LW, Nair R, Pincus T, Ringold S, Smolen JS, Stanislawska-Biernat E, Symmons D, Tak PP, Upchurch KS, Vencovský J, Wolfe F, Hawker G (2010) The 2010 American college of rheumatology/european league against rheumatism classification criteria for rheumatoid arthritis: phase 2 methodological report. Arthritis Rheum 62:2582–2591

    Article  PubMed Central  PubMed  Google Scholar 

  16. Shiboski SC, Shiboski CH, Criswell L, Baer A, et al (2012) American college of rheumatology classification criteria for Sjögren’s syndrome: A data-driven, expert consensus approach in the Sjögren’s international collaborative clinical alliance cohort. Arthritis Care Res (Hoboken), 64(4):475–487

    Google Scholar 

  17. Mosca M, Neri R, Bombardieri S (1999) Undifferentiated connective tissue diseases (UCTD): a review of the literature and a proposal for preliminary classification criteria. Clin Exp Rheumatol 17:615–620

    CAS  PubMed  Google Scholar 

  18. Jennette JC, Olson LJ, Schwartz MM, Silva GF (2007) Heptinstall’s pathology of the kidney, 6th edn. Wolters Kluwer and Lippincott Williams & Wilkins, Philadelphia

  19. Ramos-Casals M, Stone JH, Cid MC, Bosch X (2012) The cryoglobulinaemias. Lancet 379:348–360

    Article  CAS  PubMed  Google Scholar 

  20. Tzioufas A, Manoussakis M, Costello R, Silis M, Papadopoulos N, Moutsopoulos H (1986) Cryoglobulinemia in autoimmune rheumatic diseases. Evidence of circulating monoclonal cryoglobulins in patients with primary Sjögren’s syndrome. Arthritis Rheum 29:1098–1104

    Article  CAS  PubMed  Google Scholar 

  21. Wolfe F, Fries JF (2003) Rate of death due to leukemia/lymphoma in patients with rheumatoid arthritis. Arthritis Rheum 48:2694–2695

    Article  PubMed  Google Scholar 

  22. Zintzaras E, Voulgarelis M, Moutsopoulos H (2005) The risk of lymphoma development in autoimmune diseases: a meta-analysis. Arch Intern Med 165:2337–2344

    Article  PubMed  Google Scholar 

Download references

Conflict of interest

Authors do not have any conflict of interest.

Author information

Authors and Affiliations

  1. Mayo Clinic, Rochester, MN, USA

    Ladan Zand, Fernando C. Fervenza, Samih H. Nasr & Sanjeev Sethi

Authors
  1. Ladan Zand
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Fernando C. Fervenza
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Samih H. Nasr
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Sanjeev Sethi
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sanjeev Sethi.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Zand, L., Fervenza, F.C., Nasr, S.H. et al. Membranoproliferative glomerulonephritis associated with autoimmune diseases. J Nephrol 27, 165–171 (2014). https://doi.org/10.1007/s40620-014-0049-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s40620-014-0049-0

Keywords

Search

Navigation