Interstitial pneumonia with autoimmune features: A case series and overview

The topic of interstitial pneumonia with autoimmune features (IPAF) is a research classification proposed by the European Respiratory Society/American Thoracic Society Task Force: this includes patients with idiopathic interstitial pneumonia (IIP) and clinical features, suggesting an underlying auto...

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Main Authors: Ana Luísa Fernandes, Jorge Ferreira, Inês Neves
Format: Article
Language:English
Published: Elsevier 2021-01-01
Series:Respiratory Medicine Case Reports
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2213007121000125
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author Ana Luísa Fernandes
Jorge Ferreira
Inês Neves
author_facet Ana Luísa Fernandes
Jorge Ferreira
Inês Neves
author_sort Ana Luísa Fernandes
collection DOAJ
description The topic of interstitial pneumonia with autoimmune features (IPAF) is a research classification proposed by the European Respiratory Society/American Thoracic Society Task Force: this includes patients with idiopathic interstitial pneumonia (IIP) and clinical features, suggesting an underlying autoimmune process, but who do not meet established criteria for a connective tissue disease (CTD). We aimed to perform a detailed characterization of clinical, serological, and radiological features for our patients with IPAF criteria. Six patients were included, and a comprehensive description of these cases revealed a heterogeneous group in terms of clinical and treatment options. In most patients, it was possible to identify other features and disorders with an autoimmune “background,” which may support the inclusion of these patients in the IPAF classification. No deaths or significant decline in lung function occurred, and thus no definitive diagnosis of CTD could be found over 35 months of median follow-up. Therefore, IPAF is a recent concept, with many questions still open in regard to its usage in the ILD field.
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spelling doaj.art-5e614010d7014af39162b54d1479a0cd2022-12-21T21:24:34ZengElsevierRespiratory Medicine Case Reports2213-00712021-01-0132101350Interstitial pneumonia with autoimmune features: A case series and overviewAna Luísa Fernandes0Jorge Ferreira1Inês Neves2Corresponding author. , Rua Dr. Eduardo Torres / 4464-513, Senhora da Hora, Matosinhos, Portugal.; Pulmonology Department, Hospital Pedro Hispano, Matosinhos, PortugalPulmonology Department, Hospital Pedro Hispano, Matosinhos, PortugalPulmonology Department, Hospital Pedro Hispano, Matosinhos, PortugalThe topic of interstitial pneumonia with autoimmune features (IPAF) is a research classification proposed by the European Respiratory Society/American Thoracic Society Task Force: this includes patients with idiopathic interstitial pneumonia (IIP) and clinical features, suggesting an underlying autoimmune process, but who do not meet established criteria for a connective tissue disease (CTD). We aimed to perform a detailed characterization of clinical, serological, and radiological features for our patients with IPAF criteria. Six patients were included, and a comprehensive description of these cases revealed a heterogeneous group in terms of clinical and treatment options. In most patients, it was possible to identify other features and disorders with an autoimmune “background,” which may support the inclusion of these patients in the IPAF classification. No deaths or significant decline in lung function occurred, and thus no definitive diagnosis of CTD could be found over 35 months of median follow-up. Therefore, IPAF is a recent concept, with many questions still open in regard to its usage in the ILD field.http://www.sciencedirect.com/science/article/pii/S2213007121000125Interstitial pneumonia with autoimmune featuresInterstitial lung diseaseConnective tissue disease
spellingShingle Ana Luísa Fernandes
Jorge Ferreira
Inês Neves
Interstitial pneumonia with autoimmune features: A case series and overview
Respiratory Medicine Case Reports
Interstitial pneumonia with autoimmune features
Interstitial lung disease
Connective tissue disease
title Interstitial pneumonia with autoimmune features: A case series and overview
title_full Interstitial pneumonia with autoimmune features: A case series and overview
title_fullStr Interstitial pneumonia with autoimmune features: A case series and overview
title_full_unstemmed Interstitial pneumonia with autoimmune features: A case series and overview
title_short Interstitial pneumonia with autoimmune features: A case series and overview
title_sort interstitial pneumonia with autoimmune features a case series and overview
topic Interstitial pneumonia with autoimmune features
Interstitial lung disease
Connective tissue disease
url http://www.sciencedirect.com/science/article/pii/S2213007121000125
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