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Cardiopulmonary Manifestations of Collagen Vascular Diseases

  • Imaging (D Mintz, Section Editor)
  • Published:
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Abstract

Purpose of Review

The study aimed to illustrate the cardiopulmonary findings of the following collagen vascular diseases on cross-sectional imaging: rheumatoid arthritis, scleroderma (progressive systemic sclerosis), systemic lupus erythematosus, the inflammatory myopathies (polymyositis/dermatomyositis), and Sjögren’s syndrome.

Recent Findings

Although collagen vascular diseases can affect any part of the body, interstitial lung disease and pulmonary hypertension are the two most important cardiopulmonary complications and are responsible for the majority of morbidity and mortality in this patient population. Interstitial pneumonia with autoimmune features (IPAF) is a newly described entity that encompasses interstitial lung disease in patients with clinical, serologic, or morphologic features suggestive of but not diagnostic of collagen vascular disease; these patients are thought to have better outcomes than idiopathic interstitial pneumonias.

Summary

Interstitial lung disease and pulmonary hypertension determine the prognosis in collagen vascular disease patients. IPAF is a new term to label patients with possible collagen vascular disease-related interstitial lung disease. Collagen vascular disease patients are at increased risk for various malignancies.

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Authors and Affiliations

  1. Division of Diagnostic Radiology, Cardiothoracic Imaging Section, Mallinckrodt Institute of Radiology, Washington University in St. Louis, Campus Box 8131, 510 S Kingshighway Blvd, St. Louis, MO, USA

    Hamza Jawad, Sebastian R. McWilliams & Sanjeev Bhalla

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  1. Hamza Jawad
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  2. Sebastian R. McWilliams
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Correspondence to Hamza Jawad.

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Drs. Hamza Jawad, Sebastian McWilliams, and Sanjeev Bhalla declare that they have no conflict of interest.

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Jawad, H., McWilliams, S.R. & Bhalla, S. Cardiopulmonary Manifestations of Collagen Vascular Diseases. Curr Rheumatol Rep 19, 71 (2017). https://doi.org/10.1007/s11926-017-0697-x

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