3 years ago

Atteintes pulmonaires des myopathies inflammatoires

Baptiste Hervier, Raphael Borie

Publication date: June 2018

Source: Revue du Rhumatisme Monographies, Volume 85, Issue 3

Author(s): Baptiste Hervier, Raphael Borie

Résumé

Les atteintes pulmonaires survenant au cours des myopathies inflammatoires sont fréquentes et de nature multiple. Elles peuvent être spécifiques ou consécutives à l’atteinte musculaire. Leur expression clinique et leur sévérité sont variables d’un patient à l’autre et d’un sous-type de myopathie inflammatoire à un autre. Le plus souvent, ces manifestations aggravent le pronostic des patients et sont parfois même la principale cause de morbi-mortalité. C’est le cas notamment des pneumopathies interstitielles diffuses, qui dominent ces tableaux musculo-pulmonaires et dont l’évolutivité est difficile à prédire initialement. Le diagnostic, l’évaluation répétée et surtout la prise en charge spécifique de ces manifestations extramusculaire sont donc importants et requièrent un avis spécialisé. Les traitements de ces manifestations et en particulier des pneumopathies interstitielles diffuses sont urgents et reposent sur l’association de corticoïdes et d’immunosuppresseurs classiques (cyclophosphamide, azathioprine…), de plus en plus remplacés ou associés à des biothérapies (rituximab…). La place respective de chacun de traitements est cependant mal codifiée et repose actuellement essentiellement sur des avis d’experts. Ces dernières années, la découverte de nouveaux auto-anticorps spécifiques a permis une meilleure description de chacun des phénotypes des myopathies inflammatoires et de leur histoire naturelle. Les enjeux futurs sont la mise en place d’évaluations thérapeutiques rigoureuses. Ces dernières sont nécessaires pour préciser les bénéfices et les risques des différents traitements immunomodulateurs.

Abstract

Pulmonary manifestations of inflammatory myopathies are frequent and of a multiple nature. They may be specific or consecutive to muscle damage. Their clinical expression and severity vary from one patient to another and from one type of inflammatory myopathy to another. Most often, these manifestations worsen the prognosis and are frequently the main cause of morbidity and mortality, particularly in case of interstitial lung diseases. Interstitial lung diseases are the most frequent manifestations, and their evolution is difficult to predict at diagnosis. Diagnosis, evaluation and management of these manifestations are therefore important and require a specialized advice. The treatment of these manifestations and in particular in case of interstitial lung disease is urgent and is based on the combination of corticoids and classical immunosuppressants (cyclophosphamide, azathioprine…), increasingly replaced or associated with biotherapies (rituximab…). The respective place of each treatment is poorly codified however, and is currently based mainly on expert advice. While the last few years have allowed the discovery of many myositis-specific auto-antibodies and a better description of each inflammatory myopathy phenotype natural history, the future challenges concern therapeutic evaluations. There is an urgent need to clarify the benefits and risks of different immunomodulatory treatments.

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