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Case Reports
. 2021 Jan 20:32:101350.
doi: 10.1016/j.rmcr.2021.101350. eCollection 2021.

Interstitial pneumonia with autoimmune features: A case series and overview

Ana Luísa Fernandes  1 , Jorge Ferreira  1 , Inês Neves  1
Affiliations
Case Reports

Interstitial pneumonia with autoimmune features: A case series and overview

Ana Luísa Fernandes et al. Respir Med Case Rep. .

Abstract

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.

Keywords: Connective tissue disease; Interstitial lung disease; Interstitial pneumonia with autoimmune features.

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Conflict of interest statement

All authors report no conflict of interest.

Figures

Fig. 1
Fig. 1
–Radiological features of IPAF patients. (A) Fibrotic non-specific interstitial pneumonia (NSIP) with traction bronchiectasis – patient one; (B) Fibrotic non-specific interstitial pneumonia (NSIP) + Organizing pneumonia pattern – patient two; (C) Usual interstitial pneumonia pattern – patient three; (D) Fibrotic non-specific interstitial pneumonia (NSIP) – patient four; (E) Organizing pneumonia pattern – patient five; (F) Honeycombing and ground-glass on the lung bases – patient six (G) “Straight edge” sign – isolation of fibrosis to the lung bases with sharp demarcation in the craniocaudal plane without substantial extension along the lateral margins of the lungs on coronal images, more typical of ILD-CTD [13,14]. - patient one.
See this image and copyright information in PMC

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References

    1. Travis W.D., Costabel U., Hansell D.M. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am. J. Respir. Crit. Care Med. 2013;188:733–748. doi: 10.1164/rccm.201308-1483ST. - DOI - PMC - PubMed
    1. Omote N., Taniguchi H., Kondoh Y. Lung-dominant connective tissue disease: clinical, radiologic and histologic features. Chest. 2015;148:1438–1446. doi: 10.1378/chest.14-3174. - DOI - PubMed
    1. Corte T.J., Copley S.J., Desai S.R. Significance of connective tissue disease features in idiopathic interstitial pneumonia. Eur. Respir. J. 2012;39:661–668. doi: 10.1183/09031936.00174910. - DOI - PubMed
    1. Vij R., Noth I., Strek M.E. Autoimmune-featured interstitial lung disease; a distinct entity. Chest. 2011;140(5):1292–1299. doi: 10.1378/chest.10-2662. - DOI - PMC - PubMed
    1. Fischer A., Antoniou K.M., Brown K.K. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur. Respir. J. 2015;46:976–987. doi: 10.1183/13993003.00150-2015. - DOI - PubMed

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