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• W2912605389 abstract "We appreciate the constructive feedback from Drs Oldham and Danoff1Oldham J.M. Danoff S.K. Counterpoint: Does interstitial pneumonia with autoimmune features represent a distinct class of patients with idiopathic interstitial pneumonia? No.Chest. 2019; 155: 260-263Scopus (8) Google Scholar with respect to ways to refine the existing classification criteria of interstitial pneumonia with autoimmune features (IPAF). However, the charge of this pro-con debate was not whether the criteria were reflective of a distinct entity, rather that the diagnosis and concept of IPAF represented a distinct entity.2Lee J.S. Fischer A. Point: Does interstitial pneumonia with autoimmune features represent a distinct class of patients with idiopathic interstitial pneumonia? Yes.Chest. 2019; 155: 258-260Scopus (7) Google Scholar Indeed, in the original research statement, the Task Force that formulated the IPAF criteria readily acknowledged that “we must accept that in the absence of data to inform decision-making, we were left to devise what this panel believes to be a reasonable first draft of criteria that can be readily applied by investigators who wish to study this interesting—and presently poorly defined—group of patients. We recognize that the proposed criteria must be tested and validated in future studies—revisions will be needed. We are offering these criteria as a structured framework that can be applied in a uniform manner and revisited in the future.”3Fischer A. Antoniou K.M. Brown K.K. et al.An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features.Eur Respir J. 2015; 46: 976-987Crossref PubMed Scopus (627) Google Scholar We view with enthusiasm the growing number of publications about IPAF, identifying unique cohorts from various centers around the world in whom this uniform classification construct has been applied, and allowing for dedicated research of a previously amorphous cohort. These were precisely some of the goals set forth by the Task Force with its creation of the IPAF nomenclature and construct. Moreover, as acknowledged in an expert commentary, the IPAF criteria have an intrinsically changing structure and perhaps “the most important effect of these criteria is the identification of a grey zone of not well-defined rheumatology conditions.”4Cavagna L. Gonzalez Gay M.A. Allanore Y. Matucci-Cerinic M. Interstitial pneumonia with autoimmune features: a new classification still on the move.Eur Respir Rev. 2018; 27 (pii: 180047)Crossref PubMed Scopus (16) Google Scholar This spirited pro-con debate is not to determine whether the Task Force got it “right”; rather, it is about whether these types of patients represent a unique entity within the idiopathic interstitial pneumonias. We maintain that there are patients with a subset of interstitial lung disease (ILD) that has an autoimmune flavor and yet is distinct from established or well-characterized connective tissue disease-associated ILD (CTD-ILD). Without the IPAF construct, these patients had been lost among the larger framework of ILD. Furthermore, the presence of disease heterogeneity does not make IPAF any less of a distinct entity. In fact, several other ILDs that we readily recognize as distinct disease entities, such as hypersensitivity pneumonitis or CTD-ILD, have varied patterns of lung injury and natural history. There is an appreciation that heterogeneity is a reality for many forms of ILD, and IPAF is no different. We maintain that IPAF identifies a distinct class of patients within the ILD framework residing in the intersection between idiopathic interstitial pneumonia and CTD-ILD. IPAF, while originally proposed as a research construct, is evolving into a clinical diagnosis. However, without question, the first iteration of the proposed classification criteria is just a start, and revisions are needed. We are optimistic that the past, ongoing, and future research studies—as well as this type of spirited debate among multidisciplinary colleagues—will assist in enhancing the IPAF criteria and help us better understand this unique subset of ILD. COUNTERPOINT: Does Interstitial Pneumonia With Autoimmune Features Represent a Distinct Class of Patients With Idiopathic Interstitial Pneumonia? NoCHESTVol. 155Issue 2PreviewWhat's in a name? that which we call a rose By any other name would smell as sweet. William Shakespeare, Romeo and Juliet1 Full-Text PDF POINT: Does Interstitial Pneumonia With Autoimmune Features Represent a Distinct Class of Patients With Idiopathic Interstitial Pneumonia? YesCHESTVol. 155Issue 2PreviewInterstitial pneumonia with autoimmune features (IPAF) defines a distinct subset of patients with an idiopathic interstitial pneumonia (IIP). The following case from our clinic supports our position and should resonate among those who routinely evaluate patients with an interstitial lung disease (ILD). Full-Text PDF Rebuttal From Drs Oldham and DanoffCHESTVol. 155Issue 2PreviewWe agree with Drs Lee and Fischer that patients like the one described in their vignette1 have sufficient features to warrant a provisional diagnosis of unclassified connective tissue disease (CTD)-interstitial lung disease (ILD), an entity now widely referred to as interstitial pneumonia with autoimmune features (IPAF). In that respect, the IPAF research statement2 has accomplished one of its primary goals, which was to provide a single nomenclature for such patients. Unfortunately, these criteria have resulted in the lumping of patients with likely unrelated disorders into a single group. Full-Text PDF" @default.
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• W2912605389 title "Rebuttal From Drs Lee and Fischer" @default.
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