1319 - Systemic Capillary Leak Syndrome: A Case Report

A 50-year-old male with systemic capillary leak syndrome.

Case Description: This case study highlights a 50-year-old male with fatigue, hypotension, and low-grade fever following a Boy Scouts excursion. Initial concern was for septic shock due to tick-borne illness. Workup was significant for elevated hemoglobin, hypoalbuminemia, and a low-level monoclonal gammopathy. Initial cultures and infectious workups were negative. He was treated with IV fluids and required pressors. Evaluation involved multiple specialties, with hematology suspecting systemic capillary leak syndrome (SCLS). Patient’s hospital course was complicated by cardiac arrest, renal failure requiring dialysis, fungemia, four extremity compartment syndrome requiring fasciotomies, and amputation. Upon a similar presentation years later, he was treated with IVIG and was discharged from the hospital a couple of days later without significant complications.

Discussions:

SCLS is characterized by transient, severe episodes of hypotension, hemoconcentration, and hypoalbuminemia due to leakage of fluids into tissues. It is thought to be due to inflammatory stimuli causing weakening of junctions that hold epithelial cells together, leading to increased permeability. Between 2013 and 2018, there were only 134 new cases of SCLS identified. Most commonly it affects middle-aged, Caucasian adults. The female-to-male ratio is about 1:1.

Treatment involves maintaining normotension to prevent multiorgan dysfunction and then diuresis during the post-leak phase to prevent pulmonary complications. IVIG has emerged as a promising prophylactic and possibly acute-phase treatment, although its mechanism remains unclear. Early administration during the patient’s second episode appeared to improve outcomes compared to his initial course, which was marked by extensive complications.

Conclusions: This case highlights the diagnostic challenges and severe complications of SCLS. Timely recognition and consideration of IVIG may significantly alter outcomes. Greater awareness and research are needed to refine diagnostic criteria and establish evidence-based management guidelines for both acute and prophylactic treatment of SCLS.