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Abstract: PO1745

Eculizumab Use in Scleroderma Renal Crisis with Thrombotic Microangiopathy

Session Information

Category: Trainee Case Report

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Avillach, Claire, Boston Medical Center, Boston, Massachusetts, United States
  • Jaberi, Aala, Boston Medical Center, Boston, Massachusetts, United States
  • Beck, Laurence H., Boston Medical Center, Boston, Massachusetts, United States
  • Francis, Jean M., Boston Medical Center, Boston, Massachusetts, United States

Group or Team Name

  • Boston Medical Center
Introduction

Scleroderma renal crisis is a life-threatening condition with increased mortality and morbidity leading to end-stage renal disease in about 25% of cases.Here we report a case of newly diagnosed scleroderma renal crisis with thrombotic microangiopathy (TMA) successfully treated with eculizumab.

Case Description

A 44-year-old African American female with no significant past medical history, presented with acute pulmonary edema in the setting of malignant hypertension and severe accelerated acute kidney injury (AKI) with rapidly declining GFR and clinical features of systemic sclerosis sine scleroderma. Patient was commenced on renin-angiotensin system blockade (ACEI). Despite good blood pressure control on ACEI, her creatinine continued to rise with persistent hemolysis. The kidney biopsy revealed severe features of scleroderma renal crisis with active thrombotic microangiopathy (TMA), and the decision was made to treat the patient with Eculizumab in addition to ACEI. Kidney function rapidely improved on treatment from a peak creatinine of 9.5mg/dl to 2.5mg/dl over few weeks without requiring dialysis, combined to resolution of all TMA features. Eculizumab was stopped after 3 months, without sign of relapse 2 months after discontinuation.

Discussion

TMA is a common feature of SRC, reported in about 50% of cases. The positive C4d staining on the kidney biopsy in our patient supports complement activation through the classical or mannan-binding lectin (MBL) pathway. Several studies identified the presence of auto-antibodies directed against endothelial cells that could lead to complement activation . Presence of autoantibodies against vascular receptor (against angiotensin II type 1 receptor and endothelin-1 type A receptor) have been associated with pulmonary hypertension, pulmonary fibrosis and digital ulcers but no association with SRC and TMA has been reported yet. On the other hand, hemodynamic shear stress itself has been shown to activate the classical pathway, and trigger secondary TMA. In addition, an increased FBb/FB ratio has been reported in a SRC with TMA, suggestive of subsequent recruitment of the alternative pathway through the C3b feedback cycle leading to further endothelial injury. These data support the potential role of complement blockade for the treatment of SRC with TMA.