Interstitial pneumonia with autoimmune features: a new classification still on the move

In the past decade, the attention has been focused on the relationship between interstitial lung disease (ILD) and connective tissue diseases (CTDs). The clinical impact, as well as the therapeutic approach, classification and diagnosis have been addressed. These two latter items represent an area of great interest because the underlying conditions leading to ILD and the most appropriate treatment still needs to be defined. Recently, the European Respiratory Society/American Thoracic Society proposed the interstitial pneumonia with autoimmune features (IPAF) criteria which represents the effort of pulmonologists to classify patients that may remain clinically undefined [1]. The analysis of these criteria shows that IPAF, similarly to pulmonary arterial hypertension classification [2], may cluster conditions referring to a wide spectrum of rheumatic conditions such as systemic sclerosis, myositis, rheumatoid arthritis and overlap syndromes. These diseases are characterised by highly different characteristics, evolution and therapeutic approaches. Furthermore, IPAF criteria have an intrinsically changing structure, related to the regular update of existing CTD classification criteria and to the even more common identification of new biomarkers in CTDs. By far the most important effect of these criteria is the identification of a grey zone of not well-defined rheumatology conditions, e.g. anti-synthetase syndrome (ASSD) [3]. In this issue of the European Respiratory Review, Samb...
Source: European Respiratory Review - Category: Respiratory Medicine Authors: Tags: Interstitial and orphan lung disease Editorials Source Type: research