(Clarkson disease, capillary hyperpermeability syndrome, SFC, SFCI)

Prevalence: < 1/106. The cause is unknown: a hypothesis is the modification of endothelial junctions by internalization of the cadherins. A monoclonal immunoglobulin (IgGK) is present in about 80 % of the adults contrary to children where it is absent. A few familial cases have been described.

The disease evolves in crises separated by periods of remission of variable duration or as a more chronic form

Typical presentation of crises: after a triggering event (infection, postpartum, perimenstrual period, intense physical effort):

-         prodromes: 24-48 h: non-specific general signs, weight gain, diarrhea, abdominal pain

-         state phase: 1-4 days:  generalized or segmental edema (face, limbs but not pulmonary edema), major arterial hypotension with preserved consciousness,  major hemoconcentration (Hct > 55 %) with paradoxical hypoalbuminemia (< 35 g/l), hypotension, sometimes shock; acute renal failure, rhabdomyolysis, pancreatitis. Severity is variable from 1 (hypotension) to 4 (death).

-         recovery phase: 3-15 days: rapid disappearance of edema with polyuria and normalization of blood pressure; major risk of hypervolemia with acute pulmonary edema, pericardial effusion, cardiac failure.

Chronic form: generalized edema, daily variation of weight, pleural or pericardial effusion

Treatment: monthly administration of gammaglobulins; sometimes: theophylline or terbutaline

There are secondary forms  the presentation of which is less stereotyped and the crisis in generally unique. 

Their possible causes are:

-         hematologic: lymphoproliferative disorders, graft versus host disease, macrophagocytic activation syndrome

-         drugs: IL-2, chemotherapy, interferons

-         post-traumatic situation: post-bypass, necrotizing enterocolitis

-         infections: sepsis, chlamydia pneumoniae, brucellosis, dengue fever

-         systemic diseases:  Kawasaki disease, psoriasis and its treatment, Gaucher disease

-         other: ovarian hyperstimulation syndrome

Anesthetic implications: 

-         state phase: hypotension with hypoalbuminemia. very careful volume loading (risk of worsening the edema and the recovery phase): vasopressor of choice: noradrenaline; monitor renal function; risk of thrombosis

-         recovery phase: polyuria, risk of  circulatory overload (acute pulmonary edema, pericardial effusion), risk of rhabdomyolysis with compartment syndrome, sometimes cerebral edema

-        chronic form: check for a possible pleural or pericardial effusion and the blood proteins level

References:

-         Duron L, Delestre F, Amoura Z, Arnaud L. 
Syndrome de fuite capillaire idiopathique etformes secondaires : une revue systématique de la littérature. 
Rev Méd Int 2015 ; 36 : 386-94.

-         Ledochowski S, Freichet M, Prieur C, Friggeri A, Lega J-C.
An uncommon cause of distributive shock : lessons from two consecutive cases of idiopathic systemic capillary leak syndrome (Clarkson’s disease). 
Anaesth Crit Care Pain Med 2015; 34: 251-3. 

-        Jeong E-K, Kim Y-K, Kim S-H, Lee C-H, Kim J-S.
Systemic capillary leak syndrome under general anesthesia : a case report.
Korean J Anesthesiol 2014 ; 66 : 462-6

-        Datta R, Panchamia JK.
Anesthetic considerations for a patient with chronic systemic capillary leak syndrome : a case report.
A&A Practice 2018 ; 11 : 276-8

-        Tran T-A, Filleron A, Simonin M, Corbeau P.
Idiopathic systemic capillary leak syndrome in childhood : a literature review.
J Rare Dis Res Treat 2017; 2: 52-5.

-        Reference center: Hôpital Pitié-Salpêtrière (Paris): 00-33 (0)1 42 17 80 79 

Updated: Februray 2019