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Diagnostic et prise en charge du syndrome de fuite capillaire idiopathique (SFCI)

Diagnosis and management of systemic capillary leak syndrome (SCLS)

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Médecine Intensive Réanimation

Résumé

Le syndrome de fuite capillaire idiopathique (SFCI) ou syndrome de Clarkson est une maladie rare définie par la survenue de chocs répétitifs, avec hémoconcentration et hypoprotidémie paradoxale, en présence d’une gammapathie monoclonale. La physiopathologie du SFCI n’est pas connue. Il atteint les adultes après 50 ans, avec un sex-ratio équilibré. La gammapathie monoclonale est présente chez la quasitotalité des malades, le plus souvent de type IgG. Les crises conduisent souvent les malades en réanimation. Le tableau clinique associe des signes d’hypovolémie (asthénie, lipothymie, hypotension artérielle, oligurie…) et d’oedème interstitiel (oedèmes diffus, manifestations musculaires, digestives ou myocardiques…). Les anomalies biologiques sont caractéristiques, associant une élévation marquée de l’hémoglobine et de l’hématocrite avec une baisse paradoxale des protides sanguins, en l’absence de protéinurie. L’insuffisance rénale aiguë fonctionnelle oligurique est très fréquente. Les crises sont spontanément résolutives en 48 à 72 heures. Dans les formes graves, le syndrome des loges est une complication caractéristique. Le décès des malades peut survenir lors des crises graves dans un tableau de défaillance multiviscérale. Aucun traitement n’ayant fait preuve de son efficacité à la phase aiguë, il est essentiellement symptomatique. Le remplissage vasculaire excessif semble associé à une morbimortalité importante, notamment à la survenue du syndrome des loges, et devrait donc être limité au maximum. Bien que les immunoglobulines intraveineuses soient la pierre angulaire du traitement préventif des récidives, elles sont possiblement délétères à la phase aiguë, et leur utilisation doit être prudente.

Abstract

Systemic capillary leak syndrome (SCLS), also known as Clarkson’s syndrome, is a rare disease characterized by recurrent shock with a pathognomonic profile of hemoconcentration and paradoxical hypoproteinemia in the presence of monoclonal gammopathy. Pathophysiology of SCLS remains unknown. It mostly affects adults during their fifth decade with a balanced sex ratio. A monoclonal gammopathy of IgG type can be found in almost every patient. During severe attacks, patients are usually admitted to intensive care unit with a characteristic clinical picture of acute hypovolemia (e.g., asthenia, faintness, arterial hypotension, oliguria) and interstitial edema (e.g., diffuse edema with muscular, abdominal, and myocardial involvement). Biological findings are typical with highly elevated hemoglobin/ hematocrit and paradoxical hypoprotidemia, in the absence of proteinuria. Acute pre-renal failure is a very frequent feature. SCLS attacks spontaneously resolve in 48 to 72 h. In severe cases, the most classical complication is lower-limb or even four-limb compartment syndrome. Severe attacks can lead to multiple organ failure and finally prove fatal. No treatment has proven to be effective during severe attacks and management is mainly supportive. Excessive fluid therapy seems to be associated with high morbidity, especially the occurrence of compartment syndrome, and mortality; it should therefore be limited. Intravenous immunoglobulins have proven to be effective in prevention of attacks. However, as their efficacy and safety is questionable during severe attacks, their indication should be carefully weighed.

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Abbreviations

CNIL:

Commission nationale de l’informatique et des libertés

IgA:

immunoglobuline A

IgG:

immunoglobuline G

IgIV:

immunoglobulines intraveineuses

IgM:

immunoglobuline M

SFCI:

syndrome de fuite capillaire idiopathique

SOFA:

sequential organ failure assessment

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Authors and Affiliations

  1. Service de médecine interne 2, Institut E3M, CHU La Pitié-Salpêtrière, université Paris-VI, Assistance Publique–Hôpitaux de Paris, 47-83 boulevard de l’Hôpital, F-75013, Paris, France

    M. Pineton de Chambrun & Z. Amoura

  2. Service de réanimation médicale, Institut de Cardiologie, CHU La Pitié-Salpêtrière, université Paris-VI, Assistance publique–Hôpitaux de Paris, Paris, France

    M. Pineton de Chambrun, C. -E. Luyt, N. Bréchot & A. Combes

  3. Service de médecine interne, centre hospitalier Bretagne-Atlantique, 20 boulevard Général Maurice Guillaudot, F-56000, Vannes, France

    M. Gousseff

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  1. M. Pineton de Chambrun
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  2. M. Gousseff
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  3. C. -E. Luyt
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  4. N. Bréchot
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Correspondence to M. Pineton de Chambrun or Z. Amoura.

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Pineton de Chambrun, M., Gousseff, M., Luyt, C.E. et al. Diagnostic et prise en charge du syndrome de fuite capillaire idiopathique (SFCI). Méd. Intensive Réa 26, 268–276 (2017). https://doi.org/10.1007/s13546-017-1275-y

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