Capillary leak syndrome is is a situations characterized by the escape of blood plasma through capillary walls, from the blood vessels to surrounding tissues, muscle compartments, organs or body cavities.
The idiopathic form of the syndrome is characterized by three phases 1,2:
- prodrome: non-specific symptoms (e.g. lethargy, irritability)
- fluid extravasation: generalized edema causing hypotension
- fluid recruitment: sudden cessation of extravasation resulting in resolution of generalized edema and improvement in blood pressure
The syndrome may be an isolated episode, or may recur 1,2.
It can be primary or secondary 1,2:
- primary: idiopathic systemic capillary leak syndrome (or Clarkson disease)
- secondary: witnessed in several situations inclusive of
Treatment and prognosis
Treatment an episode is usually supportive, aimed at stabilizing symptoms and preventing severe complications. This may involve airway and breathing stabilization, medications, intravenous infusion of fluids, and even blood products.
History and etymology
The primary form of initially described by Bayard Clarkson, an American physician, in 1960 3.
- 1. Dhir V, Arya V, Malav IC, Suryanarayanan BS, Gupta R, Dey AB. Idiopathic systemic capillary leak syndrome (SCLS): case report and systematic review of cases reported in the last 16 years. (2007) Internal medicine (Tokyo, Japan). 46 (12): 899-904. Pubmed
- 2. Guffroy A, Dervieux B, Gravier S, Martinez C, Deibener-Kaminsky J, Hachulla E, Michel M, Weber JC, Korganow AS, Arnaud L, Gottenberg JE, Sibilia J. Systemic capillary leak syndrome and autoimmune diseases: A case series. (2017) Seminars in arthritis and rheumatism. 46 (4): 509-512. doi:10.1016/j.semarthrit.2016.08.001 - Pubmed
- 3. Clarkson B, Thompson D, Horwith M, Luckey EH. Cyclical edema and shock due to increased capillary permeability. (1960) The American journal of medicine. 29: 193-216. Pubmed