(181) A Rare Mechanism of Acute Heart Failure in Autoimmune Disease

Introduction: Autoimmune diseases are often associated with diffuse inflammation affecting multiple organs. In certain situations, an upregulation of the inflammatory cascade can abruptly damage a specific organ resulting in poor clinical outcomes. In this case, we describe an 18-year male with systemic lupus erythematous (SLE) who developed a rapid decline in systolic function secondary to an uncontrolled and severe systemic inflammation.

Description: A 18-year-old male with SLE and ESRD was transferred to our ICU with recurrent episode of supraventricular tachycardia for 1 week. Patient presented to our ICU in respiratory failure, and rapidly progressed to mechanical ventilation. A transthoracic echocardiogram showed diffuse hypokinesis and reduced ejection fraction (EF) of 10% compared to a previous EF of 55% reported 3 months before admission. Lab results revealed troponin 305 ng/L, lactic acidosis 3.1 mmol/l and mixed venous oxygen saturation of 42%. Further lab results showed profound hematologic abnormalities (anemia, thrombocytopenia and neutropenia) suggesting a SLE flare-up versus acute viral myocarditis. Therefore, patient was started on milrinone, a pulse dose of methylprednisolone over 3 days and CVVHD for volume management. However, cardiac MRI was negative for myocarditis and additional lab result revealed a ferritin 2551 ng/ml which triggered the suspicious for macrophage activation syndrome (MAS). Bone marrow biopsy showed histiocytes with evidence of hemophagocytosis. H-score was 189 points with 80% probability of MAS. The patient was started on plasmapheresis (PLEX) and anakinra.  By day 4 of treatment, ferritin decreased to 498 ng/ml.  Repeated TTE on day 5 of treatment showed EF improved to 30% and by day 30 the EF 45%. Patient was discharged home.

Discussion: MAS is a potentially life-threatening condition commonly found in patients with autoimmune conditions, infections or malignant processes. The presence of H-Score >168 and elevated soluble-IL-2 receptor have an excellent sensitivity and specificity for the diagnosis of MAS and was relevant for the management of our patient. SLE-induced MAS resulting in cardiogenic shock has been rarely reported in the literature, therefore this case illustrate the importance of early intervention to achieve good clinical outcomes.