One of the methods of treating severe forms of systemic scleroderma is hematopoietic stem-cell autotransplantation (auto-HSCT). The article describes two clinical cases of auto-HSCT followed by rituximab (RTM) therapy in patients with diffuse systemic scleroderma with progressive interstitial lung disease (ILD), high immunological activity, high skin score, and an unfavorable prognosis. The assessment of the course of these diseases was carried out in the course of dynamic observation of patients for 10–11 years. In all cases, auto-HSCT followed by RTM made it possible to achieve remission / low activity, as well as to stabilize the progressive of ILD.