Antisynthetase syndrome

We present a case of pulmonary involvement associated to idiopathic inflammatory myopathy in a relatively homogenous clinical profile known as antisynthetase syndrome. This syndrome is characterized by myositis, arthralgias/arthritis, interstitial lung disease, fever, mechanic's hands, and Raynaud's phenomenon. Autoantibodies against aminoacyl-tRNA synthetases are associated, with anti-Jo-1 being the one most commonly found. The clinical appearance of pulmonary involvement is heterogeneous, slowly progressive or acute. Myositis precedes or is concurrent with the development of lung disease in the majority of cases, but the occurrence of lung involvement before myositis occurs in more than one third of cases. Therapy of these respiratory manifestations varies with the kind of clinical onset of the disease and with the level of functional impairment and must be personalized on the basis of clinical, functional and radiological findings of the follow up.