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Masquerading as Septic Shock: Idiopathic Systemic Capillary Leak Syndrome FREE TO VIEW

Kymberly McDonald, MD; Vanessa Yap, MD; Stephen Meng, MD; Prashant Grover, MD; Daniel Gerardi, MD
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University of Connecticut Health Center, Farmington, CT

Chest. 2014;146(4_MeetingAbstracts):295A. doi:10.1378/chest.1993879
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SESSION TITLE: Critical Care Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Systemic capillary leak syndrome (SCLS) is an extremely rare condition characterized by reversible plasma extravasation and vascular collapse (1). It is a potentially fatal disorder with “attacks” of hypovolemic shock and generalized edema in association with hemoconcentration and hypoalbuminemia (2). There is much to learn about SCLS and should be considered among the differentials in unexplained shock.

CASE PRESENTATION: A 47 yo M with a history of sarcoidosis presented to the emergency department complaining of abdominal pain and nausea. He was afebrile, tachycardic and hypotensive. He appeared diaphoretic and had a bluish-purple discoloration of his distal extremities. Laboratory studies showed leukocytosis (21.4), erythrocytosis (Hb 21.7g/dL and hct 64.8%), hypoalbuminemia (1.2g/dL) and lactic acidosis (3.3mmol/L). Chest x-ray had no acute infiltrates. The patient was started on broad-spectrum antibiotics and managed in the ICU with the presumed diagnosis of septic shock. He was refractory to aggressive intravenous fluid resuscitation and required vasopressors. The patient developed acute respiratory failure requiring intubation but unfortunately passed away shortly after. All blood, respiratory and urine cultures remained negative. An autopsy showed extensive pulmonary sarcoidosis and monoclonal gammopathy of undetermined significance. The patient had been admitted to the ICU seven months prior with an identical presentation, examination, laboratory values, and management. Adrenal insufficiency and polycythemia vera were ruled out. There was no specific etiology identified before he was discharged in stable condition at his baseline level of functioning.

DISCUSSION: The triad of hypotension, hemoconcentration and hypoalbuminemia is typical on initial presentation of SCLS (1). Its cause is unknown, although is believed to be a manifestation of transient endothelial dysfunction due to endothelial contraction, apoptosis, injury, or a combination of these (1). An estimated 79-82% of patients with SCLS have monoclonal gammopathy of unknown significance (1). The diagnosis is made clinically, excluding other causes including sepsis, anaphylaxis and adrenal insufficiency. Acute episodes are treated with judicious fluid resuscitation, namely with crystalloids. Corticosteroids, plasmapheresis and intravenous γ -globulin have proved to be successful in the acute phase as reported in case series (2).

CONCLUSIONS: SCLS is characterized by the triad of hypotension, hemoconcentration and hypoalbuminemia which reflects endothelial barrier dysfunction and profoundly reduced intravascular volume. Early recognition of the condition and diagnosis of SCLS will decrease morbidity and mortality.

Reference #1: Druey K, Greipp P. Narrative review: the systemic capillary leak syndrome. Ann Intern Med 2010;153:90-8.

Reference #2: Kapoor P, Greipp P, et al. Idiopathic systemic capillary leak syndrome (Clarkson’s disease): the Mayo clinic experience. Mayo Clin Proc 2010;85:905-12.

DISCLOSURE: The following authors have nothing to disclose: Kymberly McDonald, Vanessa Yap, Stephen Meng, Prashant Grover, Daniel Gerardi

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