As antifibrotic drugs are beneficial in IPF and may be beneficial in CTD-ILD, it is conceivable that a treatment benefit may also be found in subjects with IPAF, however results of the trials are eagerly awaited. Currently, treatment decisions in patients classified as IPAF must be based on careful evaluation of benefit: risk ratio in the individual subject and should ideally be discussed in multidisciplinary setting. Prognosis is generally intermediate between that of idiopathic pulmonary fibrosis and connective tissue disease-associated interstitial lung disease, but substantially variable according to the predominant histologic and radiologic patterns. As acknowledged by the Task Force, the proposed classification scheme of IPAF is usually a research concept that will need revision and refinement based on data to better inform prognostication and patient care. requirements, in addition to a minimum of of the following domains (Table 2): Table 2 Classification criteria for interstitial pneumonia with autoimmune features [adapted from Fischer et al. (3)]. criteria independently predicted improved survival (13). Survival studies of cohorts of patients getting together with the consensus IPAF criteria have found conflicting results. The University of Chicago pulmonary cohort found that patients classified as IPAF had shorter survival than CTD-ILD patients, but a slightly better outcome than patients with IPF (12). When patients were stratified according to the high-resolution computed tomography (HRCT) pattern, patients with non-UIP IPAF pattern had a very comparable prognosis to those with CTD-ILD, while disease progression of UIP-IPAF patients resembled that of patients with IPF. The GAP index, a score developed in IPF and based on gender, age, and lung Mouse monoclonal to SIRT1 physiology (forced vital capacity (FVC) and diffusing Olodaterol capacity of the lung for carbon monoxide) predicted mortality (12). While the presence of a clinical domain was associated with a decreased mortality risk, the serological and the morphological domains were not associated with a significant increase in mortality risk. Nevertheless, the presence of a multi-compartment feature was a strong predictor of poor outcome. Conversely, our cohort from the Claude Bernard Lyon University, France, found no significant difference in overall survival between IPAF and IPF patients (10). Amongst patients with IPAF, UIP, or non-specific interstitial pneumonia (NSIP) pattern had no significant impact on survival, while history of smoking was the only factor significantly associated with increased mortality (10). In the cohort from the University of Colorado Rheumatology Clinic, patients experienced no significant decline in FVC or death during the follow-up period. This obtaining might be attributable to favorable prognostic factors among patients recruited in the study, such as the majority of patients being never-smokers, females, and responsive to effective immunosuppressive therapy (11). In other words, it appears that cohorts from pulmonology departments may be enriched in cases of IPAF with characteristics and outcome close to those of IPF (10, 12), whereas cohorts from rheumatology departments (11) may have Olodaterol characteristics closer to those of CTD-ILD. In another study, it was observed that a radiological NSIP pattern and a higher age were associated with a poor prognosis compared to other patients classified as IPAF patients with organizing pneumonia or NSIP/organizing pneumonia overlap (16). The radiological-pathological pattern was more predictive of the prognosis than highly specific autoantibodies related to known CTDs (16). A recent study from South Korea recently confirmed that patients classified as IPAF had a 1-, 3-, and 5-year survival lower than that of CTD-ILD, and better than that of patients with IPF (with fewer acute exacerbations of fibrosis) (21). However, no significant difference in survival was found between Olodaterol patients with IPAF patients and a UIP pattern and those with IPF patients (21), as previously observed in another cohort (12). As a result of these dissimilarities, longitudinal research using ILD clusters analysis has been performed to identify clinical phenotypes and to predict outcomes. Phenotypic clusters were able to anticipate lung function deterioration and survival, independently of the primary ILD classification (22). IPAF were mostly found in two clusters with a heterogeneous clinical presentationthe cluster of younger African-American females with elevated antinuclear antibody titres and in the cluster of elderly Caucasian male smokers, with severe honeycombing (22). In a recent study (17), the presence of a UIP pattern at high resolution computed tomography and/or histopathology was associated with a poor outcome as compared to a non-UIP pattern among patients with IPAF, although in general the diagnosis of IPAF was associated with a better outcome than IPF. Similarly, Yoshimura et al. (23) found that patients with a pattern of NSIP who met criteria for IPAF had a better outcome than those with idiopathic NSIP; patients with UIP and IPAF also had a better outcome than those with IPF (idiopathic UIPno IPAF). Dai.