State of the art in interstitial pneumonia with autoimmune features: a systematic review on retrospective studies and suggestions for further advances

Abstract

@ERSpublications IPAF retrospective cohorts show numerous differences between them. We propose some ideas to improve IPAF criteria http://ow.ly/eubC30jlGJO ABSTRACT The term interstitial pneumonia with autoimmune features (IPAF) has been proposed to define patients with interstitial lung disease (ILD) associated with autoimmune signs not classifiable for connective tissue diseases (CTDs). This new definition overcomes previous nomenclatures and provides a uniform structure for prospective studies through specific classification criteria.This work evaluates the characteristics of IPAF patients reported in the literature, to highlight potential limits through a comparative analysis and to suggest better performing classification criteria.Four retrospective studies on the IPAF population have been considered. The study subjects differed in age, sex, smoking habit, ILD pattern and outcomes. Another important difference lies in the diverse items considered in the classification criteria. The retrospective design of the studies and the absence from some of them of a rheumatologist clearly involved in the diagnosis may have influenced the data, but current IPAF criteria seem to include a rather heterogeneous population. To overcome these discrepancies, this review suggests a limitation in the use of single items and the exclusion of extremely specific CTD criteria. This should avoid the definition of IPAF for those diseases at different stages or at early onset. The investigation of a functional or morphological cut-off of pulmonary involvement would be useful.

Usual interstitial pneumonia: A distinct group within interstitial pneumonia with autoimmune features?

“…3 In Kelly and Moua's study, this pattern was found in approximately every fifth patient with IPAF, while in the other studies it was even more common (up to 54% of patients). 1,2 In all published series, the average frequency of UIP findings thus far reached 45%. 1 We agree with the authors that it is unclear whether IPAF-UIP represents forme fruste IPF or a unique IPAF subcategory.…”

“…1 These results are in accordance with the data from several other studies that investigated prognostic factors in IPAF. 2 IPAF is a clinical entity that encompasses patients with idiopathic interstitial pneumonia (IIP) and features suggesting an underlying autoimmune process but not meeting the established criteria for a connective tissue disease (CTD). Unlike other IIP, UIP is not incorporated in the diagnostic criteria of IPAF.…”

Idiopathic and immune‐related pulmonary fibrosis: diagnostic and therapeutic challenges

“…This grouping remains a problem and the term can be thought of as useful, in particular in the research setting, rather than a firm disease entity. A recent review comparing four recent retrospective studies showed that populations were heterogeneous in terms of features and outcomes, and that there was variable involvement of rheumatologists in the diagnostic process and inconsistent terminology was used . Special points of concern were around the inclusion of early CTD patients, especially anti‐synthetase syndrome (potentially delaying treatment), and limited the availability of extended panels of antibodies .…”

“…15 Special points of concern were around the inclusion of early CTD patients, especially anti-synthetase syndrome (potentially delaying treatment), and limited the availability of extended panels of antibodies. 15,16 Clearly, this is a group of patients where the criteria for diagnosis will continue to evolve.…”

Possible value of antifibrotic drugs in patients with progressive fibrosing non-IPF interstitial lung diseases

“…At the time of diagnosis, a complete evaluation of medical history, serological data including autoantibodies, all comorbidities and related treatments were assessed. A rheumatologic evaluation was also required to interpret any rheumatologic sign and serological data [1,[22][23][24]. Each patient underwent high resolution computed tomography (HRCT) exams at baseline time and on followup every 6-12 months.…”