Therapeutic options for the treatment of interstitial lung diseases in systemic rheumatic diseases – systemic sclerosis as an example

Abstract

Systemic sclerosis is a multisystem autoimmune disorder characterized by widespread fibrosis, primarily of the skin but also of the lung parenchyma. Interstitial lung disease is one of the most severe manifestations of the disease and a leading cause of death among patients with systemic sclerosis. This article presents and summarizes therapeutic options for interstitial lung disease – predominantely immunosuppressives and biologics (cyclophosphamide, mycophenolate mofetil, rituximab and tocilizumab) and the drugs which remodel fibrotic tissue (antifibrotics). There is increasing evidence for a beneficial effect of combination therapy (immunosuppressive + antifibrotic) on the lung parenchyma. Recently, autologous hematopoietic stem cell transplantation seems to be a therapeutic method reserved for the treatment of progressive forms of systemic sclerosis and should be performed in medical centers with experience in transplantation medicine.

Keywords

• lung diseases
• interstitial – drug therapy
• etiology; scleroderma
• systemic – complications
• drug therapy; immnunosuppressive agents – therapeutic use; cyclophosphamide – therapeutic use; mycophenolic acid – therapeutic use; rituximab – therapeutic use; antibodies
• monoclonal
• humanized – therapeutic use; indoles – therapeutic use; pyridones – therapeutic use; hematopoietic stem cell transplantation; transplantation