title: Renal involvement in primary Sjögren's syndrome: a clinicopathologic study.
abstract: OBJECTIVE
  Renal pathology and clinical outcomes in patients with primary Sjögren's syndrome (pSS) who underwent kidney biopsy (KB) because of renal impairment are reported.
  
  
  METHODS
  Twenty-four of 7276 patients with pSS underwent KB over 40 years. Patient cases were reviewed by a renal pathologist, nephrologist, and rheumatologist. Presentation, laboratory findings, renal pathology, initial treatment, and therapeutic response were noted.
  
  
  RESULTS
  Seventeen patients (17 of 24; 71%) had acute or chronic tubulointerstitial nephritis (TIN) as the primary lesion, with chronic TIN (11 of 17; 65%) the most common presentation. Two had cryoglobulinemic GN. Two had focal segmental glomerulosclerosis. Twenty patients (83%) were initially treated with corticosteroids. In addition, three received rituximab during follow-up. Sixteen were followed after biopsy for more than 12 mo (median 76 mo; range 17 to 192), and 14 of 16 maintained or improved renal function through follow-up. Of the seven patients presenting in stage IV chronic kidney disease, none progressed to stage V with treatment.
  
  
  CONCLUSIONS
  This case series supports chronic TIN as the predominant KB finding in patients with renal involvement from pSS and illustrates diverse glomerular lesions. KB should be considered in the clinical evaluation of kidney dysfunction in pSS. Treatment with glucocorticoids or other immunosuppressive agents appears to slow progression of renal disease. Screening for renal involvement in pSS should include urinalysis, serum creatinine, and KB where indicated. KB with characteristic findings (TIN) should be considered as an additional supportive criterion to the classification criteria for pSS because it may affect management and renal outcome.
authors.affiliation: Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
authors.forename: Saugar
authors.initials: S
authors.lastname: Maripuri
authors.forename: Joseph P
authors.initials: JP
authors.lastname: Grande
authors.forename: Thomas G
authors.initials: TG
authors.lastname: Osborn
authors.forename: Fernando C
authors.initials: FC
authors.lastname: Fervenza
authors.forename: Eric L
authors.initials: EL
authors.lastname: Matteson
authors.forename: James V
authors.initials: JV
authors.lastname: Donadio
authors.forename: Marie C
authors.initials: MC
authors.lastname: Hogan
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D015415:Biomarkers / Q000097:blood / Q000652:urine; D001706:Biopsy; D003449:Cryoglobulinemia / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D018450:Disease Progression; D005260:Female; D005921:Glomerulonephritis / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D005923:Glomerulosclerosis, Focal Segmental / Q000209:etiology / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000187:drug effects / Q000473:pathology*; D007676:Kidney Failure, Chronic / Q000209:etiology* / Q000473:pathology / Q000517:prevention & control; D008297:Male; D008875:Middle Aged; D009395:Nephritis, Interstitial / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D000069283:Rituximab; D012859:Sjogren's Syndrome / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D015415:Biomarkers; D007166:Immunosuppressive Agents; D000069283:Rituximab
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