Some take-home messages from the 9th International Meeting on Pulmonary Rare Diseases and Orphan Drugs

Dissemination of updated knowledge on rare and ultra-rare respiratory diseases has always been a priority of the European Respiratory Review (ERR) [1, 2]. In this issue, we publish the first of a collection of four articles that discuss hot topics with direct practical clinical consequences. First, Mackintosh et al. [3] review the challenges and controversies (which are not few!) in "interstitial pneumonia with autoimmune features" (IPAF). Following the publication, in 2015, of the classification of IPAF proposed by the European Respiratory Society (ERS) and the American Thoracic Society (ATS) [4], a number of articles have been published on this entity which truly requires a multidisciplinary approach. Airway and vascular involvement are discussed in detail, as well as the problem of severe vasculopathy that can characterise some IPAF patients. The authors also discuss the controversial issue of the therapeutic approach for this "research entity" and state that "while identification of a histological NSIP pattern would support an immunosuppressive approach, histological confirmation of a UIP pattern would lead to a more cautious approach to immunosuppression, with frequent monitoring and earlier consideration of anti-fibrotic therapy, should there be progression" [3].
Source: European Respiratory Review - Category: Respiratory Medicine Authors: Tags: Interstitial and orphan lung disease Editorials Source Type: research