TY - JOUR T1 - Long-term macrolides in diffuse interstitial lung diseases JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW DO - 10.1183/16000617.0082-2017 VL - 26 IS - 146 SP - 170082 AU - Paola Faverio AU - Francesco Bini AU - Adriano Vaghi AU - Alberto Pesci Y1 - 2017/12/31 UR - http://err.ersjournals.com/content/26/146/170082.abstract N2 - In the present review we provide currently available evidence for the use of macrolides in the treatment of diffuse interstitial lung diseases (ILDs). Up to now, research on macrolides has mainly focused on three areas. First, macrolides have shown some promising results in cellular models and case reports as antifibrotic agents, by promoting autophagy and clearance of intracellular protein aggregates and acting as regulators of surfactant homeostasis. Secondly, macrolides have an immunomodulatory effect, which has been applied in some organising pneumonia cases. In particular, macrolides have been tested in association with systemic corticosteroids as steroid-sparing agents and alone as either first-line agents in mild cases or second-line agents where steroids were poorly tolerated or had failed. Thirdly, a recent area of research concerns the possible role of macrolides as modulators of lung microbiota and the host–microbiota interaction. This function has been particularly studied in idiopathic pulmonary fibrosis patients, in whom changes in microbiota have been proved to be associated with disease progression. However, the lack of high-quality studies makes the application of macrolide therapy in ILDs a field in which research should be conducted on a large scale.Macrolides may act as microbiota modulators as well as anti-inflammatory and antifibrotic agents in ILDs http://ow.ly/stlc30gB3je ER -