Simon Zec, MD (he/him/his)
Research Fellow, Pulmonary and Critical Care Medicine
Mayo Clinic
Rochester, Minnesota, United States
Disclosure information not submitted.
Juan Pablo Domecq Garces, MD
Assistant Professor of Medicine
Mayo Clinic College of Medicine
North Mankato, Minnesota, United States
Disclosure information not submitted.
Nitesh Jain, MBBS
Assistant Professor of Medicine
Mayo Clinic College of Medicine, United States
Disclosure information not submitted.
Syed Anjum Khan, MD
Assistant Professor of Medicine
Mayo Clinic College of Medicine, United States
Disclosure information not submitted.
Title: Idiopathic Systemic Capillary Leak Syndrome: Refractory Shock Triggered by Influenza B Infection
Case Report Body:
Introduction:
Idiopathic Systemic Capillary Leak Syndrome (ISCLS) is a rare differential disease entity for sepsis characterized by spontaneous episodes of generalized edema along with the triad of hypotension, hemoconcentration, and hypoalbuminemia without albuminuria. The leak phase is characterized by refractory distributive shock. We present the first report of ISCLS triggered by Influenza B infection.
Description:
A 68-year-old male presented to the emergency department with undifferentiated shock. He complained of a 3-day history of generalized weakness, scratchy throat, lightheadedness, and headache. On examination he was afebrile with flat neck veins, cold, mottled extremities, and tachycardia with low blood pressure (BP) 58/26 mmHg. Laboratory work-up revealed neutrophilia, elevated hemoglobin (22.7 g/dL) and hematocrit (68.3%), and a normal urinalysis. Albumin was initially unavailable. Rapid viral testing was Influenza B positive. Following aggressive 6L crystalloid resuscitation, norepinephrine was initiated, and the patient was transferred to the intensive care unit (ICU). Upon arrival, BP was 82/60 mmHg and bedside ultrasound revealed a collapsible inferior vena cava. Judicious resuscitation with 4L of crystalloid continued over the next 12 hours, eventually requiring vasopressin. Due to a high clinical suspicion for capillary leak, serum albumin levels were found to be 0.7 g/dL and 0.4 g/dL, at 7 and 10 hours after admission, respectively. Despite careful fluid replacement, the patient developed bilateral pleural effusions and respiratory failure requiring invasive mechanical ventilation. On day 2 serum albumin had increased (3 g/dL), hypotension had resolved, and vasopressor support was withdrawn. Bilateral chest tubes were placed on day 3 and montelukast was given. His ICU stay was complicated by acute kidney injury without the need for renal replacement therapy. Following tracheostomy tube placement on day 9, he was transferred to a rehabilitation unit where he was decannulated. He now receives monthly intravenous immunoglobulin and is doing well 8 years after this presentation.
Discussion:
Refractory hypotension with hemoconcentration should raise suspicion for ISCLS. This case illustrates the importance of identifying a trigger and careful fluid replacement when albumin levels are unavailable.