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Introduction: Antisynthetase syndrome (ASS) is a rare autoimmune systemic disease characterised by myositis, arthritis, Raynaud's syndrome, mechanic's hands, and interstitial lung disease. Materials and methods: We present the case of a 50-year-old patient with predominant signs of polymyositis, pulmonary interstitium involvement and positive anti-Jo1 antibodies with suspected antisynthetase syndrome. Results: He was treated with glucocorticoids and following that the treatment was continued with an additional immunosuppressive therapy drug, azathioprine, which was discontinued due to a liver lesion. The treatment was then continued with cyclophosphamide. In the later course of the disease, radiological regression of pulmonary changes was monitored, and mycophenolate mofetil was introduced as a maintenance therapy. Conclusion: If left unrecognised, ASS can lead to the development of irreversible pulmonary fibrosis. However, timely application of immunosuppressive therapy can improve the quality of life and the prognosis of patients with ASS.
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