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Significance of connective tissue diseases features in pulmonary fibrosis

Vincent Cottin
European Respiratory Review 2013 22: 273-280; DOI: 10.1183/09059180.00003013
Vincent Cottin
1Hospices Civils de Lyon, Hôpital Louis Pradel, Service de pneumologie – Centre de référence national des maladies pulmonaires rares et Centre de compétences de l'hypertension artérielle pulmonaire, Lyon, and 2Université de Lyon, Université Claude Bernard Lyon 1, INRA, UMR754 INRA-Vetagrosup EPHE IFR 128, Lyon, France.
1Hospices Civils de Lyon, Hôpital Louis Pradel, Service de pneumologie – Centre de référence national des maladies pulmonaires rares et Centre de compétences de l'hypertension artérielle pulmonaire, Lyon, and 2Université de Lyon, Université Claude Bernard Lyon 1, INRA, UMR754 INRA-Vetagrosup EPHE IFR 128, Lyon, France.
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Abstract

Interstitial lung disease (ILD) can occur in any of the connective tissue diseases (CTD) with varying frequency and severity, and an overall long-term prognosis that is less severe than that of idiopathic pulmonary fibrosis (IPF). Because ILD may be the presenting manifestation of CTD and/or the dominant manifestation of CTD, clinical extra-thoracic manifestations should be systematically considered in the diagnostic approach of ILD. When present, autoantibodies strongly contribute to the recognition and classification of the CTD.

Patients with clinical extrathoracic manifestations of CTD and/or autoantibodies (especially with a high titer and/or the antibody is considered “highly specific” of an autoimmune condition), but who do not fit with established international CTD criteria may be called undifferentiated CTD or “lung-dominant CTD”. Although it remains to be determined which combination of symptoms and serologic tests best identify the subset of patients with clinically relevant CTD features, available evidence suggests that such patients may have distinct clinical and imaging presentation and may portend a distinct clinical course. However, autoantibodies alone when present in IPF patients do not seem to impact prognosis or management. Referral to a rheumatologist and multidisciplinary discussion may contribute to management of patients with undifferentiated CTD.

Footnotes

  • Provenance: Publication of this peer-reviewed article was supported by the World Scleroderma Foundation, Switzerland (principal sponsor, European Respiratory Review issue 129).

  • Conflict of interest: Disclosures can be found alongside the online version of this article at err.ersjournals.com

  • Received May 13, 2013.
  • Accepted May 28, 2013.
  • ©ERS 2013
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Significance of connective tissue diseases features in pulmonary fibrosis
Vincent Cottin
European Respiratory Review Sep 2013, 22 (129) 273-280; DOI: 10.1183/09059180.00003013

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Significance of connective tissue diseases features in pulmonary fibrosis
Vincent Cottin
European Respiratory Review Sep 2013, 22 (129) 273-280; DOI: 10.1183/09059180.00003013
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  • Article
    • Abstract
    • Introduction
    • When should CTD be suspected?
    • Does the diagnosis of established CTD affect prognosis or management?
    • CTD features may be missed in the diagnostic approach of ILD
    • Are auto-antibody and/or limited extra-thoracic manifestations significant?
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