Systemic sclerosis (SSc) is a disease characterized by vasculopathy, fibrosis of the skin and internal organs, most often complicated by interstitial lung disease (ILD). The incidence of SSc reaches 20 cases per 1 million population per year, while changes in the lungs are found in more than half of patients and are characterized by a rapid increase in fibrosis, poorly controlled respiratory failure, and an unfavorable prognosis. It is important to trace the features of the formation and course of ILD and its relationship with occupational hazards in patients diagnosed with SSc. Dust pathology of the respiratory organs (chronic dust bronchitis and silicosis) is most typical for people with long work experience in mining. Silicosis in these patients may be accompanied by SSc with rapid progression of bronchial obstruction, restrictive disorders, as well as myocardial involvement and the development of pulmonary hypertension.