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Elsevier, Immunology and Allergy Clinics of North America, 2023

DOI: 10.1016/j.iac.2023.01.005

Elsevier, Pulmonology, 2(28), p. 113-118, 2022

DOI: 10.1016/j.pulmoe.2020.01.004

Elsevier, Clinics in Chest Medicine, 3(40), p. 617-636, 2019

DOI: 10.1016/j.ccm.2019.05.008

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Connective Tissue Disease-Associated Interstitial Lung Disease

This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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Abstract

This commentary highlights the present dilemmas surrounding the classification of a patient with interstitial pneumonia who has clinical features suggesting an associated connective tissue disease but the features fall short of a clear diagnosis of connective tissue disease-associated interstitial lung disease under the current rheumatologic classification systems. This commentary illustrates what we perceive to be the limitations in the present approach to the classification of this group of patients and discusses problems with redefining the diagnosis of undifferentiated connective tissue disease to encompass patients with interstitial pneumonia. Finally, we advocate not only for a multidisciplinary approach to evaluation, but also disease classification and offer a proposal to define them as a distinct phenotype--lung-dominant CTD--for which prognostic, therapeutic, and pathobiologic implications can be tested in future, hopefully multiinstitutional, studies.