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Kidney Week

Abstract: PO0304

No Tears, Frothy Urine, and Lots of Antibodies: Sjögren Syndrome Presenting as Tubulointerstitial Nephritis

Session Information

Category: Acute Kidney Injury

  • 101 AKI: Epidemiology, Risk Factors, and Prevention

Authors

  • Lofters, Jason, Englewood Health, Englewood, New Jersey, United States
  • Flemming, Nia, Englewood Health, Englewood, New Jersey, United States
  • Adedoyin, Oluwafeyi F., Englewood Health, Englewood, New Jersey, United States
  • Simmonds, Ro-Kaye A., Englewood Health, Englewood, New Jersey, United States
  • Makartian, Lena, Englewood Health, Englewood, New Jersey, United States
Introduction

Sjogren's syndrome(pSS) is a systemic autoimmune disease that primarily affects the exocrine glands. Renal involvement is a common finding among patients however, in select patients, worsening renal function precedes these symptoms. Associated pSS renal pathologies include tubulointerstitial nephritis (TIN), glomerulonephritis and renal tubular acidosis. Here we present an unusual case of a patient with worsening renal function as a primary presentation of Sjogren’s syndrome.

Case Description

52 year old woman with no medical illnesses presented with renal impairment and retrospectively reported fatigue, 18lbs weight loss, loss of appetite as well as dry mouth, dry eyes and dysphagia. Labs: Hb 9.4g/dL, BUN 24mg/dL, Cr 1.91mg/dL and random urine protein/creatinine ratio of 2.12mg/g without microscopic hematuria. ESR 94 mm/hr, RF 175 IU/mL and positive ANA with a speckled pattern and titre of 1:1280, normal C3/C4 levels, normal dsDNA, negative hepatitis B&C, negative basement membrane IgG antibodies, negative c-ANCA and p-ANCA. Renal ultrasound showed normal sized kidneys with normal echotexture and renal biopsy revealed interstitial nephritis with moderate to severe activity and mild chronicity.
She was diagnosed with tubulointerstitial nephropathy secondary to primary Sjogren’s syndrome and was started on 1mg/kg of prednisone along with topical agents for sicca symptoms. After 2 months labs revealed Hb 12.3g/dL, Cr 1.43mg/dL, ESR 22 mm/hr and urine protein/creatinine ratio of 0.358mg/g. She has subsequently been started on a prednisone taper to which she has responded well.

Discussion

TIN is associated with a number of systemic illnesses inclusive of pSS. Renal disease in pSS is as high as 42%, with TIN accounting for approximately 10-20% of cases. TIN usually presents after the initial diagnosis of pSS but in our patient it occurred concurrently with the diagnosis. The renal involvement in pSS is usually chronic and it typically shows monolymphocytic TIN with minimal glomerular involvement. However, there is a wide range of renal pathology including TIN to MPGN, membranous nephropathy, and focal crescentic GN. Even though renal impairment does not typically precede pSS it is imperative to biopsy as early as possible so that treatment can be initiated to prevent chronic disease.