Capillary leak syndrome overview
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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
Overview
Capillary leak syndrome is a rare medical condition characterized by self-reversing episodes during which the endothelial cells which line the capillaries are thought to separate for a few days, allowing for a leakage of fluid from the circulatory system to the interstitial space, resulting in a dangerous hypotension, hemoconcentration, and hypoalbuminemia. It is a life-threatening illness because each episode has the potential to cause damage to, or the failure of, vital organs due to limited perfusion. It is often misdiagnosed as polycythemia, polycythemia vera or sepsis.
Historical Perspective
The syndrome was first described by B. Clarkson in 1960, after whom it was later informally named. Beyond numerous case reports published since then, two comprehensive reviews of clinical and research experience were published in 2010.
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.
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. Some common ones include Adrenal insufficiency, Anaphylaxis, Carcinoid Syndrome, Chronic Heart Failure, Enteropathy, Gleich syndrome, Hereditary angioedema, Inferior vena cava syndrome, Mastocytosis, Nephrotic syndrome, Ovarian hyperstimulation syndrome, Pancreatitis, Pheochromocytoma, Primary amyloidosis, Sepsis and Toxic shock syndrome. Some other conditions that should be considered as a differential diagnosis include Differentiation syndrome, Engraftment syndrome, Hemophagocytic lymphohistiocytosis, Viral hemorrhagic fevers, Snakebite envenomation, Ricin poisoning, as well as multiple autoimmune conditions.
Epidemiology and Demographics
Between 1960 to 2006, hundred cases of systemic capillary leak syndrome were reported in the world literature.
Capillary leak syndrome has been described in children, however, it is sporadic and is diagnosed most often in previously healthy, middle-aged, Caucasian adults (median age ± S.D.: 45 ± 15 yrs; age range 5 months to 74 years).
Based of 107 cases of capillary leak syndrome where information was available, 57% of the individuals were male.
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