Case: A 45-year-old woman presents to the ER with complaints of abdominal pain, diarrhea and syncope. CT scan findings were consistent with colitis, so antibiotics were initiated. Her medical history is significant for a prior history of hospitalization for presenting symptoms of abdominal pain, emesis, syncope and hypotension that was thought to be due to idiopathic capillary leak syndrome (ISCLS), secondary to COVID Infection treated with IVIG. During this hospitalization, despite initial antimicrobial therapies, she developed hypotension and PEA arrest, so an exploratory laparotomy was done, but with no intra- abdominal findings. Given her unusual presentation and prior history, idiopathic systemic capillary leak syndrome was considered, and she was again treated with IVIG with gradual symptom resolution. She continued on monthly IVIG.
Conclusion: ISCLS is a diagnosis of exclusion characterized by recurrent episodes of severe hypotension, hypoalbuminemia and hemoconcentration, typically beginning in mid-life. After initial resuscitation and stabilization, treatment is usually focused on prevention of volume overload and delicate management of fluid status. Therapy to prevent future episodes usually consists of monthly infusions of IVIG (most reports using a dose of 2 grams per month). Patients with symptoms refractory to IVIG may benefit from treatment with terbutaline and theophylline.
