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Abstract: FR-PO539

Scleroderma Renal Crisis in a Patient with Paraneoplastic Syndrome

Session Information

Category: Trainee Case Report

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Ahmed, Ayesha, Rwjmh, Piscataway, New Jersey, United States
  • Saro-Nunez, Lilian, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, United States
  • Handelsman, Cory, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, United States
Introduction

Scleroderma renal crisis affects 5-10% of patients with systemic sclerosis, with 5-year survival rate of 59%. Anti-RNA polymerase IIIAb are associated with risk of SRC and cancer. We describe a patient with paraneoplastic scleroderma who presented with AKI and HTN after steroid use and diagnosed with SRC.

Case Description

A 55-year-old woman with h/o HTN,Endometrial cancer (3/ 2017) s/p TAH-BSO, tumor debulking, paraneoplastic scleroderma (diagnosed a month back with + autoantibodies to RNA polymerase III) was sent to ER for worsening creatinine(2 mg/dl to 4.6 mg/dl in 2 weeks). Of note patient was noted to have worsening liver enzymes 2 months back and underwent liver biopsy which showed ductopenia and fibrosis. Given suspicion of an autoimmune process prednisone was initiated.On admission,patient was in acute respiratory distress requiring intubation,hypertensive (SBP 180mmHg) and in renal failure. Diagnosis of SRC was made,Captopril was initiated and steroid was discontinued. Renal biopsy showed TMA with extensive involvement of arteries, arterioles,extensive (50%) ischemic glomerular alteration, segmental duplication of glomerular capillary wall and moderate tubular injury. No significant interstitial fibrosis/tubular atrophy. She was discharged on dialysis.

Discussion

SRC is characterized by presence of high blood pressure with variable degrees of renal insufficiency.5-20% of patients with diffuse scleroderma are at risk of developing SRC.A positive RNA Pol IIIAb, present in our patient,further increases the risk of developing renal crisis. SRC associated with paraneoplastic scleroderma is even rarer.An extensive review of the literature showed only 4 cases of SRC in patients with ovarian,lung,breast and renal carcinoma. Given that SRC is a potentially life-threatening disease, we should have a high degree of suspicion in patients with scleroderma who present with hypertension and acute renal failure.

Glomerular microthrombi