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Kidney Week

Abstract: PO1517

Crescentic Glomerulonephritis in Sjögren Syndrome

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Cho, Jake Namjik, Maine Medical Center, Portland, Maine, United States
  • Jamison, Danielle, Maine Nephrology Associates PA, Portland, Maine, United States
  • Oakley, Grant, Maine Medical Center, Portland, Maine, United States
  • DiPerna, Gary, Maine Medical Center, Portland, Maine, United States
Introduction

Sjogren’s syndrome (SS) is an infiltrative autoimmune disorder involving the parotid, lacrimal and salivary glands causing sicca syndrome. Kidney involvement is variable and most often results in tubulointerstitial nephritis. Glomerular disease is infrequent with MPGN and membranous nephropathy being most prevalent pathologically.

Case Description

Patient is a 21 year old female with SS, hypothyroidism and asthma who was being evaluated for fever and fatigue. Lab evaluation revealed acute kidney injury (AKI) with creatinine rising from 1.0 to 1.8 mg/dL over several weeks. Echocardiogram was concerning for mobile echodensity at the tricuspid valve, but transesophageal echo showed no abnormalities and evaluation for infection was negative. CT showed extensive prominent lymph nodes in the chest, abdomen, and pelvis but lymph node biopsy showed benign reactive hyperplasia without neoplasm. Renal ultrasound showed normal sized kidneys. Urinalysis was positive for trace proteinuria and sediment revealed dysmorphic RBCs. She was started on high dose prednisone and hydroxychloroquine for possible lupus nephritis but due to diagnostic uncertainty and unresolved AKI, kidney biopsy was performed.

Discussion

Kidney biopsy demonstrated plasma rich interstitial nephritis with severe tubulitis consistent with SS. Interestingly, the biopsy showed 3 active cellular crescents out of 21 glomeruli with focal crescentic GN. Tubulointerstitial nephritis is the most common renal pathology in primary SS leading to renal tubular acidosis, impaired concentrating ability and proximal tubule defects. GN in SS is rare but has been associated with membranoproliferative GN, membranous nephropathy and cryoglobulinemic GN. Crescentic GN was unexpected and the treatment plan was adapted to taper the prednisone, start on Mycophenolate Mofetil and trial on Rituximab.

Light microscopy for renal biopsy of Sjogren's case depicting interstitial nephritis and crescentic glomeruli