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Interstitial Pneumonia with Autoimmune Features: Implications for Clinical Practice

  • Interstitial Lung Disease (S Dhooria, Section Editor)
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A Correction to this article was published on 05 April 2023

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Abstract

Purpose of Review

Interstitial pneumonia with autoimmune features (IPAF) is a research term that was proposed in 2015 to uniformly define patients with an idiopathic interstitial lung disease with subtle features of underlying autoimmunity. In this article, we reviewed studies that utilized IPAF criteria and examined the implications of this classification in clinical practice.

Recent Findings

There were significant differences in the clinical characteristics and outcomes of the IPAF cohorts suggesting that the interpretation and application of the IPAF criteria are variable. Despite these differences, numerous studies noted that a substantial number of patients with IPAF developed a definite connective tissue disease, and the survival rate for IPAF is likely in-between that of idiopathic pulmonary fibrosis and connective tissue disease–related interstitial lung diseases.

Summary

Further refinement of the IPAF criteria and larger prospective studies are needed to better understand the implications of IPAF in clinical practice.

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References

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Funding

This material is the result of work supported with resources and the use of facilities at the Veterans Affairs Loma Linda Healthcare System, Loma Linda, California.

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Authors and Affiliations

  1. Division of Pulmonary, Critical Care, Allergy and Sleep Medicine, Department of Medicine, Loma Linda University Health, 11234 Anderson Street, Loma Linda, CA, 92354, USA

    Niranjan Jeganathan

  2. Department of Medicine, Loma Linda University Health, Loma Linda, CA, USA

    Derrick Cleland & Matheni Sathananthan

  3. Veterans Affairs Loma Linda Healthcare System, Loma Linda, CA, USA

    Derrick Cleland

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Cleland, D., Sathananthan, M. & Jeganathan, N. Interstitial Pneumonia with Autoimmune Features: Implications for Clinical Practice. Curr Pulmonol Rep 11, 132–150 (2022). https://doi.org/10.1007/s13665-022-00296-4

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