[Life-threatening respiratory insufficiency; rare primary manifestation of the antisynthetase syndrome].

The antisynthetase syndrome (AS) is a rare auto-immune disease characterised by inflammatory myopathies, interstitial lung disease, inflammatory arthritis, Mechanic's Hands and Raynaud phenomenon. AS infrequently presents with life-threatening lung disease as its primary or sole manifestation. By means of two clinical case reports, an overview is given of recent advances in diagnosis and treatment of AS-related interstitial lung disease. We emphasise the importance of considering AS as a differential diagnosis if lung disease does not respond to standard treatment in a relatively young patient, certainly if there is even a mild suggestion of myopathy, arthritis or cutaneous involvement.