• Anna Jakubczyc and Claus Neurohr.
• Klinik Schillerhöhe, Abteilung für Pneumologie und Beatmungsmedizin, Gerlingen, Robert-Bosch-Krankenhaus.
• Dtsch. Med. Wochenschr. 2020 Apr 1; 145 (7): 470-473.

AbstractPulmonary involvement is an important aspect in the management of rheumatic diseases. Connective tissue disease-associated interstitial lung diseases (CTD-ILD) are of particular importance, as their occurrence is often decisive for patient outcome. We use corticosteroids, immunosuppressants and eventually also biologicals in the therapy of CTD-ILD.Except for the "Scleroderma Lung Study" (SLS) I and II, which confirm the effectiveness of the immunosuppressive drugs cyclophosphamide (CYC) and mycophenolate mofetil (MMF) in pulmonary involvement of scleroderma (SSc-ILD), there is little data on the treatment of other CTD-ILD. Within the group of biologicals the use of Rituximab (RTX) increases in importance. The currently expected study results compare the efficacy of immunosuppressive drugs (in particular MMF and CYC) with RTX. Other investigated biologicals include Tocilizumab in SSc-ILD and Abatacept in pulmonary involvement in rheumatoid arthritis (RA-ILD). Autologous stem cell transplantation is a potent but potentially risky therapy for severe scleroderma.For the group of pulmonary interstitial diseases of different dignity, including CTD-ILD, with a increasingly fibrosing course despite adequate therapy, (e. g. chronic hypersensitivity pneumonitis, sarcoidosis) the term and concept of "fibrosing interstitial lung diseases with progressive phenotype" (PF-ILD) is being established. In the last months studies were published, which show a positive effect of antifibrotic drugs (Nintedanib, Pirfenidon) in such constellations. Currently a number of other studies regarding the effectiveness of antifibrotics in CTD-ILD are expected to be published. The studies in this field bring new aspects in the understanding of interstitial diseases and have the potential of expanding treatment options in CTD-ILD.The topic of CTD-ILD is a difficult, but at the same time exciting field.© Georg Thieme Verlag KG Stuttgart · New York.

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