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Interstitial Lung Disease Associated with Connective Tissue Diseases

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Lung Inflammation in Health and Disease, Volume II

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 1304))

Abstract

Pulmonary manifestations of connective tissue diseases (CTD) carry high morbidity and potential mortality, and the most serious pulmonary type is interstitial lung disease (ILD). Identifying and promptly intervening CTD-ILD with immune suppressor therapy will change the natural course of the disease resulting in survival improvement. Compared to idiopathic pulmonary fibrosis, the most common presentation of idiopathic interstitial pneumonia (IIP), CTD-ILD carries a better prognosis due to the response to immune suppressor therapy. Nonspecific interstitial pneumonia (NSIP) is the most common type of CTD-ILD that is different from the fibrotic classical presentation of IPF, known as usual interstitial pneumonia (UIP). An exception is rheumatoid arthritis that presents more frequently with UIP type. Occasionally, IPF may not have typical radiographic features of UIP, and a full assessment to differentiate IPF from CTD-ILD is necessary, including the intervention of a multidisciplinary team and the histopathology. Interstitial pneumonia with autoimmune features (IPAF) shows promising advantages to identify patients with ILD who have some features of a CTD without a defined autoimmune disease and who may benefit from immune suppressors. A composition of clinical, serological, and morphologic features in patients presenting with ILD will fulfill criteria for IPAF. In summary, the early recognition and treatment of CTD-ILD, differentiation from IPF-UIP, and identification of patients with IPAF fulfill the assessment by the clinician for an optimal care.

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Authors and Affiliations

  1. Division of Rheumatology, Department of Medicine, Albany Medical College, Albany, NY, USA

    Ruben A. Peredo

  2. Rheumatology, Alaska Native Medical Center, Anchorage, AK, USA

    Vivek Mehta

  3. Division of Pulmonary & Critical Care Medicine, Albany Medical College, Albany, NY, USA

    Scott Beegle

Authors
  1. Ruben A. Peredo
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  2. Vivek Mehta
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  3. Scott Beegle
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Correspondence to Ruben A. Peredo .

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Editors and Affiliations

  1. Department of Molecular & Cellular Physiology, Albany Medical College, Albany, NY, USA

    Yong-**ao Wang

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Peredo, R.A., Mehta, V., Beegle, S. (2021). Interstitial Lung Disease Associated with Connective Tissue Diseases. In: Wang, YX. (eds) Lung Inflammation in Health and Disease, Volume II. Advances in Experimental Medicine and Biology, vol 1304. Springer, Cham. https://doi.org/10.1007/978-3-030-68748-9_5

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