A possible role for IL-17 in Clarkson's disease.
BACKGROUND
Systemic capillary leak syndrome (SCLS) is a rare disease characterized by recurrent episodes and a triad of "leak attacks": hypovolemic shock, generalized edema, hemoconcentration and paradoxical hypoalbuminemia.
Here we report a case of pediatric idiopathic SCLS with an episode of cough and fever followed two days later by myalgia, livedo, acrocyanosis, and five days later by edema, tachycardia, hypotension, and generalized tonic-clonic seizure. Moreover, we provide evidence for an LPS-induced overproduction of interferon-gamma and interleukin-17 by the patient's peripheral blood mononuclear cells one year after the attack.


CONCLUSIONS
This observation suggests the involvement of IL-17 in the pathogenesis of this disease.
Department of Pediatrics, Bicêtre University Hospital, Assistance Publique des Hôpitaux de Paris, Le Kremlin Bicêtre, France.
Mathieu
M
Simonin
Department of Immunology, Nîmes University Hospital, Montpellier-Nîmes University, Nîmes, France.
Pierre
P
Corbeau
Intensive Care and Resuscitation Unit, Bicêtre University Hospital, Assistance Publique des Hôpitaux de Paris, Le Kremlin Bicêtre, France.
Philippe
P
Durand
La Pitié-Salpêtrière Hospital, Biotherapy (CIC-BTi) and Inflammation-Immunopathology-Biotherapy Department (I2B), Assistance Publique des Hôpitaux de Paris, Sorbonne Université, UPMC Univ Paris 06, INSERM, UMRS 959, Immunology-Immunopathology-Immunotherapy (I3), Paris, France.
Michelle
M
Rosenzwajg
Department of Pediatrics, Nîmes University Hospital, Nîmes, France; INSERM U 1183, Montpellier University, Montpellier, France.
Anne
A
Filleron
Department of Pediatrics, Nîmes University Hospital, Nîmes, France; INSERM U 1183, Montpellier University, Montpellier, France. Electronic address: tu.anh.tran@chu-nimes.fr.
Tu-Anh
TA
Tran
D019559:Capillary Leak Syndrome / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000235:genetics*; D002648:Child; D003371:Cough / Q000209:etiology; D004487:Edema / Q000209:etiology; D004830:Epilepsy, Tonic-Clonic / Q000209:etiology; D005260:Female; D005334:Fever / Q000209:etiology; D006801:Humans; D020381:Interleukin-17 / Q000235:genetics*; D007963:Leukocytes, Mononuclear / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D063806:Myalgia / Q000209:etiology; D016896:Treatment Outcome
C508717:IL17A protein, human; D020381:Interleukin-17
Children; Clarkson's disease; IL-17; Systemic capillary leak syndrome