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Abstract: FR-PO540

Systemic Sclerosis Sine Scleroderma Renal Crisis: An Atypical Presentation

Session Information

Category: Trainee Case Report

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Olalo, Princella, Harbor UCLA Medical Center, LA Biomed, Torrance, California, United States
  • Sharma, Kavita G., Harbor UCLA Medical Center, Irvine, California, United States
  • Mccurdy, Deborah K., UCLA, Los Angeles, California, United States
  • Chandramohan, Gangadarshni, Harbor UCLA Medical Center, LA Biomed, Torrance, California, United States
Introduction

Scleroderma renal crisis (SRC) is a rare complication that occurs in 5% of all systemic sclerosis (SSc) patients. Previously reported one-year mortality of 76% decreased to 15% when captopril became the preferred treatment. Usually, SRC is triggered by high dose steroids in patient with SSc. Our patient instead, presenting with signs of Hemolytic Uremic Syndrome (HUS) diverting the workup towards infectious/autoimmune/genetic causes of HUS until, renal biopsy and response to captopril became the key elements of diagnosis.

Case Description

This is a 19-year-old female presenting with vomiting, diarrhea and abdominal pain that progressed into dyspnea, oliguria, anasarca, malignant hypertension and renal failure. No evidence of skin or joint manifestations. Lab tests: low platelets, low Hg with schistocytes, stools (-)ve for E. coli and Shiga-toxin, normal ADAMTS13 activity and (-)ve Scl-70 antibodies. But, positive antinuclear antibody (ANA) and Sjogren’s antibody (SsA) implied an autoimmune process. After failing a course of steroid pulse, plasmapheresis and plasma exchange transiently increased platelets and Hg. But, anuria persisted, requiring daily dialysis. Renal biopsy: onion peel lesion in medium sized arterioles, likely scleroderma renal crisis, prompted the decision to start captopril. After one dose, she had 2 liters of urine and eventually, renal function returned to normal.

Discussion

This is the first case report of a suspected HUS, later being diagnosed as SSc sine SRC based on clinical findings, positive ANA and SsA, renal arteriolar vasculopathy and response to captopril treatment without cutaneous manifestations and negative Scl-70 panel. Thus, it is important to consider SSc sine SRC when presenting with such clinical picture to attain complete renal recovery and decrease mortality.