[Pulmonary involvement in polymyositis and dermatomyositis].
Polymyositis is characterised by an inflammatory reaction in skeletal muscle with a variable degree of muscular weakness and associated with skin lesions in the case of dermatomyositis. Involvement of the muscles of deglutition and the diaphragm may lead to inhalation pneumonia and acute or chronic respiratory failure, often hypercapnic. The other respiratory manifestations are diffuse interstitial pneumonitis (DIP), usually non-specific, and very occasionally pulmonary arterial hypertension. The development of DIP during polymyositis is a grave prognostic factor, respiratory involvement being one of the main causes of morbidity and mortality. The onset of DIP is acute in between 30 and 47% of cases. Anti-synthetase antibodies (particularly anti-JO-1) are positive in about 75% of cases. Treatment is usually with a combination of immunosuppressants and corticosteroids without any immunosuppressants therapy having shown a superiority.
Tillie-Leblond
I
I
Service de Pneumologie et d'Immuno-Allergologie, Hôpital Calmette, Lille, France. i-tillie@chru-lille.fr
Colin
G
G
Lelong
J
J
Cadranel
J
J
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000974:Antibodies, Antinuclear / Q000276:immunology; D003882:Dermatomyositis / Q000150:complications; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008171:Lung Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D017563:Lung Diseases, Interstitial / Q000209:etiology; D017285:Polymyositis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D011379:Prognosis; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D000974:Antibodies, Antinuclear; D007166:Immunosuppressive Agents; C046750:Jo-1 antibody