Skip to main content
SpringerLink
Log in

A patient presenting with isolated hematuria and renal dysfunction as rare manifestation of cryoglobulinemic glomerulonephritis in the course of autoimmune diseases including Sjögren’s syndrome

  • Case Report
  • Published:
CEN Case Reports Aims and scope Submit manuscript

Abstract

Autoimmune diseases are sometimes associated with immune-mediated renal diseases and cryoglobulinemia is one of the causes. Cryoglobulinemia and cryoglobulinemic glomerulonephritis associated with primary Sjögren’s syndrome are most frequent condition among non-hepatitis C virus-related condition. Its typical renal manifestation shows high amount of proteinuria with microscopic hematuria and renal insufficiency. We describe a case of 72-year-old woman with Hashimoto disease, autoimmune hepatitis, Sjögren’s syndrome, and immune-related pancytopenia complicated by cryoglobulinemic glomerulonephritis. Before kidney biopsy, tubulointerstitial nephritis probably due to Sjögren’s syndrome was suspected because of persistent hematuria without significant proteinuria and developing mild renal dysfunction over 6 months. The developing renal dysfunction associated with isolated hematuria is uncommon in glomerular diseases. Kidney biopsy, however, revealed established membranoproliferative glomerulonephritis with subendothelial deposits consisting of tubular structures with IgM, IgG, and C3 staining. Corticosteroids plus mycophenolate mofetil therapy successfully normalized renal function. Physician should not overlook cryoglobulinemic glomerulonephritis, which is potentially poor prognosis, even if urinalysis shows only persistent isolated hematuria in patients 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
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Matignon M, Cacoub P, Colombat M, Saadoun D, Brocheriou I, Mougenot B, Roudot-Thoraval F, Vanhille P, Moranne O, Hachulla E, Hatron PY, Fermand JP, Fakhouri F, Ronco P, Plaisier E, Grimbert P. Clinical and morphologic spectrum of renal involvement in patients with mixed cryoglobulinemia without evidence of hepatitis C virus infection. Medicine (Baltimore). 2009;88:341–8.

    Article  Google Scholar 

  2. Zaidan M, Terrier B, Pozdzik A, Frouget T, Rioux-Leclercq N, Combe C, Lepreux S, Hummel A, Noël LH, Marie I, Legallicier B, François A, Huart A, Launay D, Kaplanski G, Bridoux F, Vanhille P, Makdassi R, Augusto JF, Rouvier P, Karras A, Jouanneau C, Verpont MC, Callard P, Carrat F, Hermine O, Léger JM, Mariette X, Senet P, Saadoun D, Ronco P, Brochériou I, Cacoub P, Plaisier E. CryoVas study group. Spectrum and prognosis of noninfectious renal mixed cryoglobulinemic GN. J Am Soc Nephrol. 2016;27:1213–24.

    Article  CAS  Google Scholar 

  3. Anand A, Krishna GG, Sibley RK, Kambham N. Sjögren syndrome and cryoglobulinemic glomerulonephritis. Am J Kidney Dis. 2015;66:532–5.

    Article  Google Scholar 

  4. Monti G, Galli M, Invernizzi F, Pioltelli P, Saccardo F, Monteverde A, Pietrogrande M, Renoldi P, Bombardieri S, Bordin G, Candela M, Ferri C, Gabrielli A, Mazzaro C, Migliaresi S, Mussini C, Ossi E, Quintiliani L, Tirri G, Vacca A. Cryoglobulinaemias: a multi-centre study of the early clinical and laboratory manifestations of primary and secondary disease. GISC. Italian Group for the Study of Cryoglobulinaemias. QJM. 1995;88:115–26.

    CAS  PubMed  Google Scholar 

  5. Maripuri S, Grande JP, Osborn TG, Fervenza FC, Matteson EL, Donadio JV, Hogan MC. Renal involvement in primary Sjögren’s syndrome: a clinicopathologic study. Clin J Am Soc Nephrol. 2009;4:1423–31.

    Article  Google Scholar 

  6. Goules A, Masouridi S, Tzioufas AG, Ioannidis JP, Skopouli FN, Moutsopoulos HM. Clinically significant and biopsy-documented renal involvement in primary Sjögren syndrome. Medicine (Baltimore). 2000;79:241–9.

    Article  CAS  Google Scholar 

  7. Morimoto C, Fujigaki Y, Tamura Y, Ota T, Shibata S, Asako K, Kikuchi H, Kono H, Kondo F, Yamaguchi Y, Uchida S. Emergence of smoldering ANCA-associated glomerulonephritis during the clinical course of mixed connective tissue disease and Sjögren’s syndrome. Intern Med. 2017. https://doi.org/10.2169/internalmedicine.9844-17.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Wheeler DS, Giugliano RP, Rangaswami J. Anticoagulation-related nephropathy. J Thromb Haemost. 2016;14:461–7.

    Article  CAS  Google Scholar 

  9. Zeher M, Horvath IF, Szanto A, Szodoray P. Autoimmune thyroid diseases in a large group of Hungarian patients with primary Sjögren’s syndrome. Thyroid. 2009;19:39–45.

    Article  CAS  Google Scholar 

  10. Ramos-Casals M, Stone JH, Cid MC, Bosh X. The cryoglobulinaemias. Lancet. 2012;379:348–60.

    Article  CAS  Google Scholar 

  11. Zhao Y, Li Y, Wang L, Li XF, Huang CB, Wang GC, Zhang XW, Zhang ZL, Zhang X, Xiao WG, Dai L, Wang YF, Hu SX, Li HB, Gong L, Liu B, Sun LY, Zhang MJ, Zhang X, Li YZ, Du DS, Zhang SH, Sun YY, Zhang FC. Primary Sjögren syndrome in Han Chinese: clinical and immunological characteristics of 483 patients. Medicine (Baltimore). 2015;94:e667.

    Article  CAS  Google Scholar 

  12. Retamozo S, Gheitasi H, Quartuccio L, Kostov B, Corazza L, Bové A, Sisó-Almirall A, Gandía M, Ramos-Casals M, De Vita S, Brito-Zerón P. Cryoglobulinaemic vasculitis at diagnosis predicts mortality in primary Sjögren syndrome: analysis of 515 patients. Rheumatology. 2016;55:1443–51.

    Article  Google Scholar 

  13. Willeke P, Schlüter B, Becker H, Schotte H, Domschke W, Gaubitz M. Mycophenolate sodium treatment in patients with primary Sjögren syndrome: a pilot trial. Arthritis Res Ther. 2007;9:R115.

    Article  Google Scholar 

  14. Koratala A, Reeves WH, Segal MS. Tubulointerstitial nephritis in sjögren syndrome treated with mycophenolate mofetil. J Clin Rheumatol. 2017;23:402–3.

    Article  Google Scholar 

  15. Pereira PR, Viala K, Maisonobe T, Haroche J, Mathian A, Hié M, Amoura Z, Cohen Aubart F. Sjögren sensory neuronopathy (Sjögren Ganglionopathy): long-term outcome and treatment response in a series of 13 cases. Medicine (Baltimore). 2016;95:e3632.

    Article  Google Scholar 

  16. Colucci G, Manno C, Grandaliano G, Schena FP. Cryoglobulinemic membranoproliferative glomerulonephritis: beyond conventional therapy. Clin Nephrol. 2011;75:374–9.

    Article  CAS  Google Scholar 

  17. Gorevic PD, Kassab HJ, Levo Y, Kohn R, Meltzer M, Prose P, Franklin EC. Mixed cryoglobulinemia: Clinical aspects and long-term follow-up of 40 patients. Am J Med. 1980;69:287–308.

    Article  CAS  Google Scholar 

Download references

Acknowledgements

We would like to thank Ms. Hiromi Yamaguchi for her technical assistance.

Author information

Authors and Affiliations

  1. Department of Internal Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, Japan

    Masaki Yamanaka, Yoshihide Fujigaki, Hajime Kono, Michito Nagura, Shigeyuki Arai, Yoshifuru Tamura, Tatsuru Ota, Shigeru Shibata & Shunya Uchida

  2. Department of Pathology, Teikyo University Hospital, 2-11-1 Kaga, Itabashi-ku, Tokyo, Japan

    Fukuo Kondo & Yutaka Yamaguchi

Authors
  1. Masaki Yamanaka
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Yoshihide Fujigaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Hajime Kono
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Michito Nagura
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Shigeyuki Arai
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Yoshifuru Tamura
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Tatsuru Ota
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Shigeru Shibata
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Fukuo Kondo
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Yutaka Yamaguchi
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Shunya Uchida
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Yoshihide Fujigaki.

Ethics declarations

Conflict of interest

The authors have declared that no conflict of interest exists.

Ethical approval

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

Informed consent

Written informed consent was obtained from the patient.

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Yamanaka, M., Fujigaki, Y., Kono, H. et al. A patient presenting with isolated hematuria and renal dysfunction as rare manifestation of cryoglobulinemic glomerulonephritis in the course of autoimmune diseases including Sjögren’s syndrome. CEN Case Rep 7, 211–216 (2018). https://doi.org/10.1007/s13730-018-0329-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13730-018-0329-y

Keywords

Search

Navigation