Acta Academiae Medicinae Sinica

Acta Academiae Medicinae Sinica

Acta Academiae Medicinae Sinica ›› 2018, Vol. 40 ›› Issue (2): 268-278.doi: 10.3881/j.issn.1000-503X.2018.02.019

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Clinicopathological Features and Treatment of Renal Impair in Primary Sjögren Syndrome

WANG Jing, CHEN Limeng()   

  1. Department of Nephrology,PUMC Hospital,CAMS and PUMC,Beijing 100730,China
  • Received:2016-06-17 Online:2018-04-28 Published:2018-05-03
  • About author:The first two author contributed equally to this article

Abstract:

Primary Sjögren syndrome,characterized by autoimmune epithelitis,is a prevalent systemic autoimmune disease involving multiple organs,among which kidney is a major target organ.Tubulointerstitial lesion is the most frequent form,involving proximal tubule,distal tubule,or collecting duct.The disease has an occult onset and may progressively develop into renal function impairment and end-stage renal disease,which can be accompanied with low-molecular-weight proteinuria,renal tubule acidosis and electrolyte disturbance.Pathologically,it is featured by lymphocyte infiltration,renal tubule atrophy,and interstitial fibrosis.Glomerular lesion is less common and usually takes the form of membranoproliferative glomerulitis.Glucocorticoid combined with immunosuppresant is the main treatment option,and B cell-targeted therapy has been reported.Most patients respond well to these treatments.In this article we review the prevalence,clinicopathological features,and treatment of renal disease in primary Sjögren syndrome.

Key words: primary Sjögren syndrome;, renal tubule acidosis, nephrogenic diabetes insipidus, Fanconi syndrome, membranoproliferative glomerulitis

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