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Complications aiguës de la sclérodermie systémique

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Maladies rares en médecine d’urgence

Part of the book series: Références en médecine d’urgence. Collection de la SFMU ((REFERMED))

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Résumé

La sclérodermie systémique (ScS) est une affection généralisée du tissu conjonctif, des artérioles et des micro-vaisseaux, caractérisée par l’accumulation de matrice extracellulaire, une atteinte microvasculaire oblitérante et la présence d’anomalies de l’immunité humorale et cellulaire [1]. Elle se traduit cliniquement par des anomalies de la microcirculation dominées par un phénomène de Raynaud, des lésions de sclérose cutanée et des manifestations viscérales. Il s’agit d’une maladie rare puisque sa prévalence en France est évaluée à 158/millions d’habitants, soit un nombre de patients sclérodermiques en France de 6000 à 8000 [2]. La ScS est caractérisée par une grande variabilité dans sa présentation clinique, entrainant parfois des difficultés et des retards diagnostiques importants. Il n’est pas rare que le diagnostic soit fait à l’occasion d’une complication viscérale ou dans une situation d’urgence lors d’une décompensation de cette atteinte viscérale. L’urgentiste peut donc se trouver en première ligne pour évoquer le diagnostic. Le diagnostic repose sur des critères diagnostiques de l’American College of Rheumatology (ACR) [3] (tableau I).

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Authors and Affiliations

  1. Pôle de médecine interne, Hôpital Cochin, AP-HP et université Paris Descartes, 75679, Paris Cedex 14, France

    A. Berezne, G. Bussone & L. Mouthon

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  1. A. Berezne
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  2. G. Bussone
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  3. L. Mouthon
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Correspondence to L. Mouthon .

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Berezne, A., Bussone, G., Mouthon, L. (2013). Complications aiguës de la sclérodermie systémique. In: Maladies rares en médecine d’urgence. Références en médecine d’urgence. Collection de la SFMU. Springer, Paris. https://doi.org/10.1007/978-2-8178-0350-0_7

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  • DOI: https://doi.org/10.1007/978-2-8178-0350-0_7

  • Publisher Name: Springer, Paris

  • Print ISBN: 978-2-8178-0349-4

  • Online ISBN: 978-2-8178-0350-0

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