Idiopathic capillary leak syndrome (ICLS) occurs as a result of vascular membrane instability, which results in the leakage of several proteins from the vascular compartment to the interstitial spaces. It is an extremely rare disorder, with around 260 cases documented thus far. We present a case of a 35-year-old male with a past medical history of asthma and gastroesophageal reflux disease who initially presented to our hospital for the treatment of chronic demyelinating inflammatory neuropathy requiring plasmapheresis and steroid therapy. After removal of his vascular catheter, he experienced sudden onset of dyspnea, hypotension, and respiratory distress. His lab work showed a hemoglobin of 21.3 g/dL and a hematocrit of 62.6%. Protein electrophoresis showed a mildly decreased albumin at 3.28 g/dL. These findings were consistent with ICLS. He required management with colloids and systemic steroids. The difficult diagnosis of ICLS is due to its overlap with several medical emergencies, such as sepsis and anaphylaxis. Further studies are required to study the role of steroids and colloids in the management of ICLS.
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