Capillary leak syndrome
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: M. Hassan, M.B.B.S
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Synonyms and Keywords: Systemic capillary leak syndrome; SCLS; Clarkson's disease.
Overview
Historical Perspective
Pathophysiology
The pathogenesis of capillary leak syndrome remain unclear, however, cytokines, vascular endothelial growth factor (VEGF), leukotrienes, and complement play an important role. During each episode of hyperpermeability of the vasculature, there is an increase of interleukin 2 receptor-positive peripheral blood M-cell count, as well as apoptosis of the endothelial cells.
Causes
Differentiating Capillary leak syndrome from other Diseases
As capillary leak syndrome has overlapping clinical and laboratory findings with various other conditions, it should be considered as the diagnosis of exclusion. Several conditions that may be considered as the differential diagnoses of capillary leak syndrome include polycythemia vera, hereditary angioedema, Gleich syndrome, adrenal insufficiency, mast cell disease, acute gastroenteritis, carcinoid syndrome, primary amyloidosis, chronic heart failure, sepsis, and toxic shock syndrome.
Epidemiology and Demographics
Risk Factors
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms | Physical Examination | Laboratory Findings | Other Diagnostic Studies
Treatment
Medical Therapy | Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies