Atteintes pulmonaires des myopathies inflammatoires

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.