Anti-synthetase syndrome presenting with interstitial lung disease

Anti-synthetase syndrome is an idiopathic inflammatory myopathy characterized by interstitial lung involvement, arthritis, Raynaud's phenomenon, mechanic's hands and fever. The case of anti-synthetase syndrome in a young female is reported. The patient presented with interstitial lung disease, initially subclinical muscle involvement, Raynaud's phenomenon, arthritis and mechanic's hands. Induction therapy was administrated, that consisted of intravenous methylprednisolone and cyclophosphamide, followed by azathioprine and prednisone with good response, and finally, complete remission. The presence of mechanic's hands in a patient with lung involvement should prompt for specific investigations such as anti-synthetase antibodies and muscle enzymes. Anti-synthetase syndrome should be aggressively managed by immunosuppressive therapy, as interstitial lung disease is a severe manifestation of the disease which may affect quality of life and life expectancy.