Critical Care: Student/Resident Case Report Poster - Critical Care IV |

Recurrent Unexplained Hypotension: Consider Systemic Capillary Leak Syndrome FREE TO VIEW

Kiet Ma, DO; Kim Jordan, MD; Richard Ko, DO
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Riverside Methodist Hospital, Columbus, OH

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):447A. doi:10.1016/j.chest.2016.08.460
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SESSION TITLE: Student/Resident Case Report Poster - Critical Care IV

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Systemic capillary leak syndrome (SCLS), or Clarkson’s disease, is a rare disorder characterized by hemoconcentration, hypoalbuminemia, reversible plasma extravasation and vascular collapse. Less than 250 cases have been reported since its first description in 1960. The diagnosis may be missed as symptoms are nonspecific and mimic other disease states.

CASE PRESENTATION: A 54 year old white female was admitted with flu like symptoms, anasarca, and hypotension with a systolic blood pressure of 70 mmHg. She reported five prior episodes of severe hypotension attributed to sepsis and hypovolemia. Evaluation for adrenal insufficiency was negative. Her hemoglobin was 19.9 g/dL and albumin was 2.0 g/dL. Chest CT revealed normal lung fields. Antibiotics and stress dose steroids were initiated for possible sepsis, but blood cultures remained negative. Initial CVP was 3 cm H2O and fluid resuscitation and pressors were started. She subsequently was intubated for pulmonary edema from volume overload. IVIG 1g daily and albumin 25g every 8 hours were started for possible SCLS. A monoclonal IgG kappa band was detected. She experienced rapid improvement, was extubated and weaned from pressors by day 4. She was discharged by day 8 on monthly IVIG treatment.

DISCUSSION: SCLS affects both genders and all ages. Patients experience recurrent acute hypotension, anasarca, hypoalbuminemia, polycythemia, and paraproteinemia, predominantly IgG kappa light chains. Exact cause is currently unknown. Paraproteins may play an immunoregulatory role; 82% of SCLS patients present with a monoclonal gammopathy. SCLS stages include: 1) prodromal phase with upper respiratory flu-like symptoms, 2) acute leak phase with increased capillary permeability and fluid and protein extravasation causing edema and hypotension, and 3) the post leak phase with recruitment of fluid back into the vascular space. In one case series, 75% of deaths were related to hypoperfusion during the acute leak phase and fluid overload during the post leak phase. Complications include compartment syndrome, renal failure, and thrombosis. Treatment data is limited by disease rarity, but hemodynamic stability is achieved with IV fluids, pressors, albumin, and IVIG during the acute leak phase. Epoprostenol, immune modulation therapy, rituximab, infliximab, plasmapheresis, and steroids have been tried, but data is limited. Maintenance theophylline, terbutaline, and IVIG may decrease future exacerbations.

CONCLUSIONS: Clinicians must consider SCLS in cases of recurrent hypotension and edema in order to provide prompt and appropriate management and prevent mortality.

Reference #1: Marra AM, Gigante A, Rosato E. Intravenous immunoglobulin in systemic capillary leak syndrome: a case report and review of literature, Expert Rev Clin Immunol 2014.10:3, 349-352.

Reference #2: Druey, Kirk M.; Greipp, Philip R. Narrative Review: Clarkson Disease-Systemic Capillary Leak Syndrome. Ann Intern Med 2010. 153 (2): 90-8.

DISCLOSURE: The following authors have nothing to disclose: Kiet Ma, Kim Jordan, Richard Ko

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