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Archives de pédiatrie
Volume 22, n° 12
pages 1263-1267 (décembre 2015)
Doi : 10.1016/j.arcped.2015.09.007
Received : 28 November 2014 ;  accepted : 13 September 2015
Dermatomyosite juvénile et nouveaux auto-anticorps : à propos de quatre observations
Juvenile dermatomyositis and new autoantibodies: Cases and review
 

M. Guarella a, , A.-L. Jurquet a, K. Retornaz a, N. Bardin b, M.-C. Chastang c, M. Desjonquere c, N. Fabien d, A. Belot c
a Service de médecine infantile, hôpital Nord, chemin des Bourrely, 13015 Marseille, France 
b Laboratoire d’immunologie, hôpital La-Conception, 147, boulevard Baille, 13005 Marseille, France 
c Service de néphrologie, rhumatologie et dermatologie pédiatriques, hôpital Femme-Mère-Enfant, université Lyon, 159, boulevard Pinel, 69677 Bron, France 
d Laboratoire d’immunologie, centre hospitalier Lyon Sud, Lyon, France 

Auteur correspondant.
Résumé

La dermatomyosite juvénile (DMJ) est la plus fréquente des myopathies inflammatoires de l’enfant. Son diagnostic est essentiellement clinique et repose sur les critères de Bohan et Peter (1975). Plus récemment, des auto-anticorps spécifiques des myosites (MSA) ont pu être associés chez l’adulte à des formes évolutives particulières. Leur valeur diagnostique et pronostique reste à déterminer chez l’enfant. Nous rapportons 4 cas de DMJ avec MSA dont nous décrivons les caractéristiques cliniques, biologiques et radiologiques ainsi que les traitements utilisés. L’étude immunologique a montré la présence d’un anticorps (Ac) anti-MDA5 (ou CADM 140), un anti-TIF-1-γ (ou p155/140) et 2 anti-NXP2 (ou p140/MJ). Les 4 enfants avaient une atteinte cutanée typique ou sévère, en particulier celui ayant des Ac anti-TIF-1-gamma qui avait présenté des lésions initialement urticariformes avec un œdème important des paupières, puis des lésions de vascularite nécrotique au niveau du décolleté. Ils ont tous présenté une atteinte musculaire initiale sauf celui ayant des Ac anti-MDA 5 qui a eu une atteinte musculaire secondaire avec un taux de créatine-phosphokinase (CPK) normal, la particularité de son tableau étant marquée par une pancréatite aiguë grave. Ces nouveaux auto-Ac spécifiques pourraient devenir des outils diagnostiques et thérapeutiques mais également des paramètres pronostiques déterminants.

The full text of this article is available in PDF format.
Summary

Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in children. Its diagnosis is usually made on a clinical basis following the criteria of Bohan and Peter (1975). Recently, the presence of myositis-specific autoantibodies (MSAs) have started to be associated with specific outcome in adult patients; the diagnosis and prognosis value of these autoantibodies remains to be identified in children. We report four cases of JDM with MSAs focusing on clinical, biological, and radiological manifestations, and then we describe associated treatment. The cohort comprises four girls with an average age of 8.5 years. The time to diagnosis was 1 week to 4 months. For these patients, the immunologic study found one patient positive for the MDA5 antibody (or CADM 140), one positive for the TIF1γ antibody (or p155/140), and two patients positive for the NXP2 antibody (or p140/MJ). Each patient showed specific and characteristic cutaneous manifestations. For example, the girl positive for the TIF1γ antibody presented the most severe skin disease with urticaria, face edema, and vascularity of the neck and shoulders. However, regarding muscular features, proximal weakness was present in most of the cohort, except for the child positive for the MDA5 antibody, who presented no sign of muscular disease at the beginning with low CK levels. Importantly, acute pancreatitis also affected this patient. Concerning radiological indications, muscular MRI evidenced hyperinflammation, a sign of diffuse myositis, in all these patients. Treatments consisted in corticosteroids together with methotrexate or mycofenolate mofetil associated or not with intravenous immunoglobulin therapy. This report highlights the importance of systematic detection and analysis of MSA in diagnosis and characterization of JDM, and describes a new approach that would allow more focused treatments and be a useful predictor of clinical complications and prognosis in JDM-affected subjects.

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