title: Renal involvement in primary Sjögren syndrome.
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.
authors.affiliation: 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, 94275 Le Kremlin-Bicêtre, Paris, France.
authors.forename: Hélène
authors.initials: H
authors.lastname: François
authors.affiliation: 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, 94275 Le Kremlin-Bicêtre, Paris, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Mariette
mesh_terms: D006801:Humans; D007674:Kidney Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D009395:Nephritis, Interstitial / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D015995:Prevalence; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis