Capillary leak syndrome
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Synonyms and Keywords: Systemic capillary leak syndrome; SCLS; Clarkson's disease.
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 (low blood pressure), 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,[1] 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.[2][3]
Natural History
The episode usually consists of two phases
1. The capillary leak phase (1-4 days) The initial phase is the capillary leak phase, lasting from 1 to 4 days. Clinical features are abdominal pain, nausea, generalized edema and hypotension that may result in cardiopulmonary collapse. Acute renal failure is due to acute tubular necrosis consequent to hypovolemia and rhabdomyolysis.
2. Recruitment of the interstitial fluid The second phase results in the recruitment of the initially extravasated fluid. Intravascular overload with polyuria and pulmonary edema often occur. Edema may be more severe due to massive fluid supply in the initial phase. It's necessary to monitor the patient in order to switch to depletion treatment with diuretics or hemofiltration.
Prognosis
Mortality is reported in 21% of the 57 cases described. However, better management of this condition has recently led to lower mortality.
In the Mayo Clinic’s experience, the median survival of 25 patients that were followed over 30 years (counting only SCLS-related deaths) was approximately 15 years, and their 5-year survival rate was 76%.[2][3] In European experience, the 5-year post-diagnosis survival rate was 85% in 23 patients who had received prophylactic treatment and 20% in 5 patients who had not.[4] However, better identification and management of this condition appears to be resulting in lower mortality and improving survival and quality-of-life results as of late.
Diagnosis
Symptoms
Generalized swelling may be present.
Physical Examination
Vitals
Skin
Laboratory Studies
- Hemoconcentration
- Hypoalbuminemia without albuminuria
Case Reports
Sources
- Dorlands Medical Dictionary 30th Ed.
- Cancer.gov
- Orphanet (2003)
References
- ↑ Clarkson, Bayard; Thompson, David; Horwith, Melvin; Luckey, E.Hugh (1960). "Cyclical edema and shock due to increased capillary permeability". The American Journal of Medicine. 29 (2): 193–216. doi:10.1016/0002-9343(60)90018-8. PMID 13693909.
- ↑ 2.0 2.1 Druey, Kirk M.; Greipp, Philip R. (2010). "Narrative Review: Clarkson Disease-Systemic Capillary Leak Syndrome". Annals of Internal Medicine. 153 (2): 90–8. doi:10.1059/0003-4819-153-2-201007200-00005. PMC 3017349. PMID 20643990.
- ↑ 3.0 3.1 Kapoor, Prashant; Greipp, Patricia T.; Schaefer, Eric W.; Mandrekar, Sumithra J.; Kamal, Arif H.; Gonzalez-Paz, Natalia C.; Kumar, Shaji; Greipp, Philip R. (2010). "Idiopathic Systemic Capillary Leak Syndrome (Clarkson's Disease): The Mayo Clinic Experience". Mayo Clinic Proceedings. 85 (10): 905–12. doi:10.4065/mcp.2010.0159. PMC 2947962. PMID 20634497.
- ↑