Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disease characterized by recurrent episodes of acute life-threatening attacks of shock, hemoconcentration, and hypoalbuminemia. Increase in capillary permeability results in reversible plasma movement into the interstitial spaces followed by appearance of related symptoms or complications, including renal failure. This condition can be potentially life-threatening; however, it is easily misdiagnosed.
A 47-year-old man with no previous medical history presented to the emergency department after experiencing general weakness and abdominal pain. He develops hypovolemic shock within 3 h of presentation and initial laboratory tests showed hemoconcentration, hypoalbuminemia and acute kidney injury. Following vigorous fluid therapy and supportive care, the patient recovers, but a similar episode recurrs after 4 months without any specific trigger. Based on the combined clinical manifestations and laboratory findings of both the attacks, he is diagnosed with ISCLS. Symptomatic relief is achieved via oxygen supplementation and massive volume replacement using normal saline and the patient is prescribed bambuterol 10 mg and theophylline 400 mg once-a-day. He is discharged from the hospital on day 5 of hospitalization. Thereafter, the patient is being followed for 5 years without any symptoms or recurrence of ISCLS even in the situation of COVID-19 infection.
ISCLS is an extremely infrequent and commonly misdiagnosed disease. However, early diagnosis, treatment and prophylaxis through accumulated clinical data can prevent ISCLS recurrence and the development of related fatal complications. Therefore, clinicians need to be well aware of the variety of clinical characteristics and treatment options of this disease. Read the full article here.