Idiopathic Systemic Capillary Leak Syndrome: A Clinical Case.

Area of Science:

• Vascular Biology
• Hematology
• Rare Diseases

Background:

• Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disorder characterized by increased vascular permeability, leading to hypotension, hypoalbuminemia, and edema.
• The exact etiopathogenesis of ISCLS remains unknown, but it is frequently associated with monoclonal gammopathy, present in over 80% of cases.
• Current management focuses on supportive care to maintain blood pressure and organ perfusion during acute crises.

Observation:

• A 72-year-old male presented with generalized edema and significant fluid effusions (pleural, pericardial, peritoneal).
• Laboratory findings revealed severe hypoalbuminemia, hypoproteinemia, and immunoglobulin G kappa monoclonal gammopathy.
• Exclusion of other causes of anasarca confirmed the diagnosis of ISCLS associated with monoclonal gammopathy.

Findings:

• The patient initially responded to albumin and diuretic therapy, showing clinical improvement.
• Despite initial response, the patient was readmitted with severe hypotension and multiorgan dysfunction.
• The patient unfortunately succumbed to the condition shortly after readmission.

Implications:

• This case underscores the critical association between ISCLS and monoclonal gammopathy.
• It highlights the challenges in managing ISCLS, even with supportive therapies.
• Further research into targeted treatments for ISCLS, particularly in the context of monoclonal gammopathy, is warranted.