We present a challenging clinical case of an antisynthetase syndrome (ASS) with a four-year follow-up. The disease debuted with skin manifestations and interstitial lung disease (ILD), then the severe Raynaud's phenomenon came to the fore with the development of occlusive vasculopathy and critical digital ischemia. After the relief of vascular lesions, the severity of the condition was determined by ILD. The use of combined pulse therapy with cyclophosphamide and methylprednisolone, treatment with intravenous immunoglobulin made it possible to reduce the activity of ASS: lung lesion and the progression of vasculopathy. However, after the termination of an unplanned pregnancy, the patient again experienced an exacerbation with ILD progression. It was decided to use rituximab, against which the patient's condition was stabilized. Clinical and laboratory remission was achieved, which was maintained for a year and a half. However, the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic triggered a re-exacerbation of the pulmonary domain of the disease, which forced us to use a nintedanib with a positive clinical and instrumental effect.