A significant progress has been made in recent years in management of severe systemic scleroderma (SSD) manifestations, such as Raynaud’s phenomenon, renal crisis, and pulmonary arterial hypertension, subsequently improving survival and quality of life. At the same time, treatment algorithms for interstitial lung damage in SSD have not yet been developed. The review provides relevant information on therapeutic efficacy of drugs with various mechanisms of action, including immunosuppressive drugs (cyclophosphamide, mycophenolate mofetil, etc.), and high-dose chemotherapy followed by autologous hematopoietic stem cell transplantation. New drugs with antifibrotic activity, including recently marketed in Russia nintedanib for treatment of interstitial lung diseases in SSD, as well as perspectives for potential use of biologics (rituximab, tocilizumab, etc.), and lung transplantation are considered separately.