ABNORMAL PRESENTATION OF INFLUENZA A-RELATED SYSTEMIC CAPILLARY LEAK SYNDROME

Abstract

SESSION TITLE: Case Report Semifinalists 9 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Systemic Capillary Leak Syndrome (SCLS) is a rare and life threatening disorder that can present as fluid-resistant shock in the presence of hemoconcentration, hypotension, and hypoalbuminemia. To date, no cause of SCLS has been confirmed, and mainstays of therapy have been supportive care and IVIG based on limited case reports. CASE PRESENTATION: A 41 year-old man presented with lower extremity swelling and malaise and profound hypotension after a three day viral prodrome. This patient was hospitalized within the past year for similar symptoms of shock with hemoconcentration and hypoalbuminemia without clear etiology. Upon arrival, patient had hypotension resistant to aggressive fluid resuscitation, followed by rapidly progressing renal and cardiac failure and eventually requiring endotracheal intubation due to laryngeal edema. Patient received intravenous immunoglobulin (IVIG) shortly thereafter for possible SCLS. During resuscitation, patient had increasingly tight muscular compartments concerning for compartment syndrome; patient was evaluated by orthopedics and was taken to operating theatre where he received bilateral upper and lower extremity fasciotomies of legs, thighs, forearms. It is known that SCLS presents with a “leak phase” during which extravasation and shock is common, followed by a “post-leak phase” during which extravasated fluid rapidly moves intravascularly. To avoid further compartment syndrome and post-leak volume overload, a goal of normotension with a central venous pressure of 4-8 mmHg was successfully used to guide resuscitation. Due to association of SCLS with oncologic processes, urgent hematology and oncology consult was obtained. Patient underwent evaluation for leukemia and lymphoma without any evidence of malignancy. During this time, patient tested positive for influenza A via PCR. Patient was noted upon workup to have elevated levels of IL-6. The patient was discharged on hospital day 15 to sub-acute rehab with instructions to receive tocilizumab, a biologic targeted at IL-6, if they experienced similar edema and malaise. DISCUSSION: There are scant case reports suggesting influenza A as the inciting factor for SCLS exacerbation. However, this is the first reported case of influenza-associated SCLS with an elevated interleukin-6 level, suggesting a clinical hypothesis that IL-6 may play a role in SCLS. Furthermore, very few of influenza-related SCLS have presented with such acuity, making this a very unusual case. CONCLUSIONS: Difficult to diagnose and manage, SCLS remains poorly understood. Little is known about best management, although IVIG and guided resuscitation may have a role. In this patient, early diagnosis and intervention led to a favorable outcome. Furthermore, an elevated in IL-6 in this patient invokes speculation towards a possible therapy. Reference #1: Ebdrup L, Druey K, Mogensen TH Severe capillary leak syndrome with cardiac arrest triggered by influenza virus infection Case Reports 2018;2018:bcr-2018-226108. Reference #2: Kapoor P, Greipp PT, Schaefer EW, Mandrekar SJ, Kamal AH, Gonzalez-Paz NC, Kumar S, Greipp PR. Idiopathic systemic capillary leak syndrome (Clarkson's disease): the Mayo clinic experience. Mayo Clin Proc. 2010 Oct;85(10):905-12. https://doi.org/10.4065/mcp.2010.0159. Epub 2010 Jul 15. PubMed PMID: 20634497; PubMed Central PMCID: PMC2947962. DISCLOSURES: No relevant relationships by Alexander Croft, source=Web Response No relevant relationships by Rajat Kapoor, source=Web Response