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Kidney Week

Abstract: PO0286

A Rare Case of Evans Syndrome with Systemic Lupus Erythematosus and Pulmonary Nocardiosis

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical, Outcomes, and Trials

Author

  • Kanipakam, Reddappa Venkata Sai Rakesh, Brown University, Providence, Rhode Island, United States
Introduction

Evans syndrome (ES) is a rare autoimmune disorder characterized by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune-mediated thrombocytopenic purpura (ITP). The exact pathophysiology is unknown but is initiated by having autoantibodies attacking body's own red blood cells and platelets.

Case Description

20-year-old male with history of autoimmune dysregulation including SLE (diagnosed when he was 13), Evan's Syndrome, class 5 lupus nephritis presented to the Hospital for difficulty breathing, dry cough and 30-pound weight loss. He was admitted to ICU for acute hypoxic respiratory failure requiring intubation. His lab work showed anemia, thrombocytopenia, AKI, ANA+, low C3, C4, elevated LDH, high uric acid, UA showing 3+ blood and 6 grams of proteinuria. A chest CT on admission also noted extensive LAD, splenomegaly, ground glass opacities and interstitial prominence in lungs. He underwent a lung biopsy which showed DAH. The patient was also treated with pulsed steroids, plasmapheresis and Cytoxan. He also had MSSA bacteremia treated with antibiotics and rehab course complicated with pulmonary nocardiosis.

Discussion

Although ES is an extremely rare case, it is important to keep broad differentials as renal dysfunction is common from different pathologies. Usually the most common cause of AKI associated with ES is ATN from intravascular hemolysis and had widespread intratubular hemoglobin casts but it is essential to consider other etiologies as in our case of previous lupus flare.