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Evaluation of Lung Disease in Patients with Connective Tissue Disease

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Pulmonary Manifestations of Rheumatic Disease

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Abstract

Lung disease is a common manifestation of connective tissue disease (CTD) and is associated with significant morbidity and mortality. The evaluation of lung disease, and interstitial lung disease (ILD) in particular, in patients with CTD is complex because of the heterogeneity of the CTDs and the varied types and degrees of severity of ILD encountered and because ILD can be identified at any point in time in these patients. A thorough—and multidisciplinary—evaluation is needed when CTD patients develop ILD or when evaluating ILD patients for the presence of occult CTD. Determining that ILD is associated with an established CTD requires the exclusion of alternative etiologies and thorough assessments of the clinical features of both the CTD and ILD. The detection of occult CTD in patients with so-called “idiopathic” ILD requires careful attention to the demographic profile, historical clues, subtle physical examination findings, specific autoantibody positivity, and radiologic and histopathologic features and can be optimized by a multidisciplinary approach that includes rheumatologic collaboration. A standardized assessment with the serial implementation of objective tests to determine disease severity and evidence of progression is important for the longitudinal monitoring of patients with CTD-associated ILD and helps guide management considerations.

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Author information

Authors and Affiliations

  1. Division of Rheumatology, Department of Medicine, Autoimmune Lung Center, National Jewish Health, 1400 Jackson Street G07, Denver, CO, 80206, USA

    Aryeh Fischer M.D.

  2. Department of Medicine, National Jewish Health, 1400 Jackson Street, Denver, CO, 80401, USA

    Kevin K. Brown M.D.

Authors
  1. Aryeh Fischer M.D.
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  2. Kevin K. Brown M.D.
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Corresponding author

Correspondence to Aryeh Fischer M.D. .

Editor information

Editors and Affiliations

  1. Harvard Medical School, Department of Medicine, Division of Rheumatology Brigham and Women’s Hospital, Boston, Massachusetts, USA

    Paul F. Dellaripa

  2. Division of Rheumatology, Autoimmune and Interstitial Lung Disease Program, Department of Medicine, National Jewish Health, Denver, Colorado, USA

    Aryeh Fischer

  3. Department of Internal Medicine, Division of Pulmonary/Critical Care Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA

    Kevin R. Flaherty

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Fischer, A., Brown, K.K. (2014). Evaluation of Lung Disease in Patients with Connective Tissue Disease. In: Dellaripa, P., Fischer, A., Flaherty, K. (eds) Pulmonary Manifestations of Rheumatic Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-0770-0_2

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  • DOI: https://doi.org/10.1007/978-1-4939-0770-0_2

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4939-0769-4

  • Online ISBN: 978-1-4939-0770-0

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