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Antisynthetase syndrome (ASS) is characterized by myositis, interstitial lung disease, Raynaud’s phenomenon, fever and mechanics hands. Diagnosis is confirmed with the detection of an antibody directed against anti–aminoacyl–transfer–RNA synthetases (ARS). The most common anti–ARS antibody is anti–Jo–1. Opportunistic infections are common causes of mortality in patients with autoimmune diseases. Immunosuppressive treatment further contributes to the risk of infection. We report a rare case of a 68 year–old man diagnosed with antisynthetase syndrome associated to a pulmonary tuberculosis infection, revealed with an acute respiratory failure. The diagnosis of this rare combination of a connective tissue disease and tuberculosis revealed with an acute respiratory failure is difficult in a previously asymptomatic patient. Early diagnosis and immunosuppressive therapy associated to antituberculosis treatment started precociously prevented the disease progression and resulted in a good outcome.