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  • Review Article
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Renal involvement in primary Sjögren syndrome

Nature Reviews Nephrology volume 12, pages 82–93 (2016)Cite this article

Subjects

Key Points

  • Renal involvement in primary Sjögren syndrome (pSS) is a rare complication that occurs in <10% patients and usually has a favourable prognosis

  • Tubulointerstitial nephritis (TIN) is the most frequent renal complication of pSS, and is characterized by infiltration of the interstitium with plasma cells and lymphocytes

  • Patients with pSS and TIN usually respond to steroid therapy alone; immunosuppressive agents and/or B-cell targeted therapies might enable steroid sparing but their use remains to be established

  • Patients with pSS, cryoglobulinaemia and low serum complement levels have a high risk of developing lymphoma and/or membranoproliferative glomerulonephritis, which responds well to steroid treatment plus rituximab and plasma exchange

  • Renal disease in pSS is often pauci-symptomatic; appropriate biologic screening of the serum and urine is required to detect and prevent terminal chronic kidney disease

  • Identification of renal disease in suspected pSS should prompt an investigation to exclude other inflammatory disorders, such as lupus erythematosus, hepatitis C virus infection and IgG4-associated disorders

Abstract

Primary Sjögren syndrome (pSS) is an autoimmune disorder characterized by lymphoplasmacytic infiltration of the exocrine (salivary and lachrymal) glands that results in sicca symptoms (dryness of the eyes and mouth). Systemic complications can occur in pSS, but renal involvement is rare, affecting <10% patients. The most frequent form of nephropathy in pSS is tubulointerstitial nephritis (TIN), in which infiltration of the kidney by plasma cells is a key feature and shows similarity to the lymphoplasmacytic infiltration of the salivary glands. Electrolyte disturbances may occur in pSS, such as renal distal tubular acidosis, diabetes insipidus, Gitelman syndrome or Fanconi syndrome. Glomerular involvement is less frequently detected in patients with pSS, but usually takes the form of membranoproliferative glomerulonephritis secondary to cryoglobulinaemia. The renal prognosis in patients with pSS and TIN or glomerular disease is usually favourable, but the risk of chronic kidney disease remains high in patients with TIN. Appropriate screening must be performed at least once a year in patients with systemic pSS in order to facilitate the early detection of renal complications. In this Review we discuss the epidemiology, pathophysiology, differential diagnosis and treatment of renal disease in pSS.

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Figure 1: Possible mechanisms involved in the pathophysiology of renal disease during primary Sjögren syndrome.
Figure 2: Typical histologic presentation of tubulointerstitial nephritis in a patient with pSS.
Figure 3: Typical histologic presentation of MPGN secondary to cryoglobulinaemia in a patient with pSS.

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Acknowledgements

The authors would like to thank Dr Charlotte Mussini (Department of Pathology, Bicetre Hospital, Assistance Publique-Hôpitaux de Paris) for providing the histologic images and Dr Magali Jasiek (Nephrology Department, Bicetre Hospital, Assistance Publique-Hôpitaux de Paris) for her contribution to Tables 2 and 3.

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Authors and Affiliations

  1. Department of Nephrology, Dialysis, and Transplantation, Hôpitaux Universitaires Paris-Sud, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris-Saclay, 78 rue du Général Leclerc, Paris, 94275, Le Kremlin-Bicêtre, France

    Hélène François

  2. Department of Rheumatology, Hôpitaux Universitaires Paris-Sud, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris-Saclay, IMVA Centre of Immunology of Viral Infections and Autoimmune Diseases, 78 rue du Général Leclerc, Paris, 94275, Le Kremlin-Bicêtre, France

    Xavier Mariette

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H.F. and X.M. researched the data for the article, provided a substantial contribution to discussions of the content, contributed equally to writing the article and to review and/or editing of the manuscript before submission.

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Correspondence to Xavier Mariette.

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François, H., Mariette, X. Renal involvement in primary Sjögren syndrome. Nat Rev Nephrol 12, 82–93 (2016). https://doi.org/10.1038/nrneph.2015.174

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