Critical Care |

Cytokine Storm: Adult-Onset Still's Disease (AOSD), Myocarditis, and Hemophagocytosis FREE TO VIEW

Atul Palkar, MD; Sonu Sahni, MD; Margarita Oks, MD; Arunabh Talwar, MD; Eric Gottesman, MD
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Hofstra-North Shore-LIJ School of Medicine, New Hyde Park, NY

Chest. 2015;148(4_MeetingAbstracts):214A. doi:10.1378/chest.2277413
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SESSION TITLE: Critical Care Cases III

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Wednesday, October 28, 2015 at 11:00 AM - 12:15 PM

INTRODUCTION: AOSD is a rare idiopathic systemic inflammatory disorder seen in young adults.

CASE PRESENTATION: A 38 year old woman with prior AOSD in remission and off immunosuppression presented with jaw pain for two weeks, fever, myalgia, and arthralgia for one day. She was hypotensive on admission and developed acute respiratory failure after fluid resuscitation requiring endotracheal intubation, mechanical ventilation and vasopressor support. She was initiated on antibiotics. Serum procalcitonin level was 26 ng/mL. She required continuous veno-venous hemodialysis for acute renal failure. Peak serum troponin level was 5.41 ng/mL. Transthoracic echocardiogram revealed severe global left ventricular systolic dysfunction. Dobutamine was added. She continued to spike fever. Infectious workup was unrevealing. Serum ferritin was 24606 ng/mL and serum triglyceride was 479 mg/dL. She was initiated on high dose intravenous steroids for AOSD flare. Serial echocardiograms showed improvement in left ventricular function that allowed extubation. Later she developed pancytopenia. A bone marrow biopsy showed hemophagocytosis and confirmed hemophagocytic lymphohistiocytosis (HLH). Anakinra was initiated for persistent pancytopenia. Pancytopenia and fever resolved prior to discharge.

DISCUSSION: Our patient had AOSD flare complicated by myocarditis and HLH. Serum procalcitonin is a poor predictor of infection in AOSD. Myocarditis in AOSD is an under-recognized complication. Refractory cases may warrant treatment with Anakinra, Infliximab, Tocilizumab or intravenous immunoglobulin. HLH is associated with infections, lymphoma, inflammatory disorders and may require bone marrow transplantation. HLH and AOSD often have overlapping features. The presence of pancytopenia and hemophagocytosis on bone marrow exam favors HLH. High serum ferritin level is typical of AOSD. Nevertheless, serum ferritin level >10,000ng/mL is more suggestive of HLH complicating AOSD as in our patient.

CONCLUSIONS: It is essential to identify myocarditis and HLH in a patient with AOSD. Early recognition of these entities is crucial to limit associated high mortality.

Reference #1: Gerfaud-Valentin M,et al. Adult-onset Still's disease. Autoimmunity Reviews. 2014;13(7):708-22.

Reference #2: Scirè CA, et al. Diagnostic value of procalcitonin measurement in febrile patients with systemic autoimmune diseases. Clin Exp Rheumatol 2006;24(2):123-8.

Reference #3: Fautrel G, et al. Diagnostic value of ferritin and glycosylated ferritin in adult onset Still’s disease, Journal of Rheumatology, 2001;28(2):322-9.

DISCLOSURE: The following authors have nothing to disclose: Atul Palkar, Sonu Sahni, Margarita Oks, Arunabh Talwar, Eric Gottesman

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