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Cardiopulmonary Manifestations of Collagen Vascular Diseases
Hamza Jawad1, Sebastian R McWilliams2, Sanjeev Bhalla2
1Division 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. jawadh@wustl.edu.
Insights
Collagen vascular diseases commonly cause interstitial lung disease and pulmonary hypertension, impacting patient prognosis. A new classification, interstitial pneumonia with autoimmune features (IPAF), identifies patients with better outcomes than idiopathic lung disease.
Area of Science:
- Cardiopulmonary imaging
- Rheumatology
- Immunology
Background:
- Collagen vascular diseases (CVDs) encompass a group of autoimmune disorders affecting connective tissues.
- These conditions can manifest with diverse systemic manifestations, including significant cardiopulmonary involvement.
Purpose of the Study:
- To illustrate the cross-sectional imaging findings of cardiopulmonary complications in specific CVDs.
- To highlight rheumatoid arthritis, scleroderma, systemic lupus erythematosus, inflammatory myopathies, and Sjögren's syndrome.
Main Methods:
- Review of cross-sectional imaging findings in patients with diagnosed collagen vascular diseases.
- Analysis of literature focusing on cardiopulmonary manifestations.
Main Results:
- Interstitial lung disease (ILD) and pulmonary hypertension are the most critical cardiopulmonary complications, driving morbidity and mortality in CVDs.
- Interstitial pneumonia with autoimmune features (IPAF) is a newly recognized entity for ILD in patients with suggestive autoimmune features, offering potentially better prognoses than idiopathic ILDs.
- CVD patients face an elevated risk of malignancies.
Conclusions:
- Interstitial lung disease and pulmonary hypertension are key determinants of prognosis in patients with collagen vascular diseases.
- IPAF represents a significant advancement in classifying and managing ILD within the spectrum of autoimmune diseases.
- Early recognition and management of cardiopulmonary complications are crucial for improving outcomes in CVD patients.
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. 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.