Interstitial pneumonia with autoimmune features.

Interstitial pneumonia with autoimmune features. J R Coll Physicians Edinb. 2020 Sep;50(3):247-255 Authors: Santhanam S, Mohanasundaram K, Krishnan S Abstract Interstitial lung disease (ILD) is an umbrella term for lung disease characterised by inflammation and fibrosis of the interstitium. ILD can be idiopathic or secondary to connective tissue disorders, drugs or environmental exposures. Before labelling it as idiopathic we have to rule out secondary causes. ILD is one of the most common extra-articular manifestations of connective tissue diseases (CTDs), causing significant morbidity and mortality. Patients with pre-existing CTD can develop ILD; some patients develop ILD against the background of either one or two clinical features of a CTD or isolated auto-antibody positivity. The current terminology for such an entity is interstitial pneumonia with autoimmune features (IPAF). The current criterion is based on three domains: clinical, serologic and morphologic. To satisfy the IPAF classification criteria, one needs to satisfy the mandatory criterion with one feature from two of the three domains. Classifying patients with this criterion helps in early initiation of immunosuppression and in monitoring them closely for development of features of a well defined CTD. There are a few limitations like the clinical domain being more skewed towards systemic sclerosis and in˜flammatory myositis, exclusion of antineutrophilic cytoplasmic ...
Source: Journal of the Royal College of Physicians of Edinburgh - Category: General Medicine Tags: J R Coll Physicians Edinb Source Type: research