Le syndrome SAPHO

Resume L'acronyme « SAPHO » a ete defini pour designer un ensemble de pathologies du systeme osteoarticulaire associe a des manifestations cutanees. Il s'agit de : la Synovite, l' Acne (acne conglobata ou fulminans), la Pustulose palmo-plantaire souvent psoriasique, l'Hyperostose et l' Osteite. Comme defini par Kahn et al. en 1994, les criteres diagnostiques du Sapho sont au nombre de trois : 1) la presence d'une osteomyelite multifocale sterile, avec ou sans lesions cutanees; 2) une atteinte articulaire aigue ou chronique associee a une pustulose palmoplantaire, un psoriasis pustuleux palmoplantaire, un acne severe, ou une hidrosadenite (de Verneuil); 3) Une osteite mono ou polyostotique sterile associee a une pustulose palmoplantaire, un psoriasis pustuleux palmoplantaire, un acne severe. La presence d'un seul de ces criteres suffit pour le diagnostic. L'evolution clinique se fait le plus souvent sur un mode chronique, neanmoins les symptomes peuvent parfois regresser spontanement, il n’ y a jamais de lesions malignes ou septiques. Le diagnostic de chaque cas doit prendre en compte tous les elements cliniques, radiologiques et histologiques; d'autant plus que l'apparition des signes dermatologiques n'est pas necessairement concomitante a ceux osseux. Les causes du syndrome SAPHO sont encore pratiquement inconnues. Compte tenu de la diversite syndrome, il apparait evident qu'il ne peut y avoir une seule etiologie. L'incidence et la prevalence ne sont pas disponibles, faute de donnees internationales. La frequence de l'OMCR (Osteomyelite multifocale chronique recidivante, une des frequentes manifestations du SAPHO) est estimee 0,04% en Allemagne. Il n'existe pas de traitement codifie pour le syndrome SAPHO. Le traitement symptomatique est base sur les AINS (anti-inflammatoire non steroidien) ou, les sulfamides tels la sulfasalazine. La calcitonine ou les biphosphonates peuvent etre proposes du fait de leur tropisme osseux. Par analogie avec la spondylarthrite ankylosante, quelques essais menes avec differents traitements immunomodulateurs, dont les anti-TNF-alpha ont ete effectues. Dans la plupart des cas, le methotrexate est administre. Les succes sont difficiles a evaluer, faute d'etude controle stricte. Notre equipe associe deux voies therapeutiques : des antiinflammatoires ou immunomodulateurs (Azithromycine ) et des hormones osteotropes (la calcitonine).*

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