TY - JOUR
T1 - Cardiopulmonary Manifestations of Collagen Vascular Diseases
AU - Jawad, Hamza
AU - McWilliams, Sebastian R.
AU - Bhalla, Sanjeev
N1 - Publisher Copyright:
© 2017, Springer Science+Business Media, LLC.
PY - 2017/11/1
Y1 - 2017/11/1
N2 - 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.
AB - 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.
KW - Cardiac magnetic resonance imaging
KW - Collagen vascular diseases
KW - Connective tissue diseases
KW - Interstitial lung disease
KW - Pulmonary hypertension
UR - http://www.scopus.com/inward/record.url?scp=85031793712&partnerID=8YFLogxK
U2 - 10.1007/s11926-017-0697-x
DO - 10.1007/s11926-017-0697-x
M3 - Review article
C2 - 28994016
AN - SCOPUS:85031793712
VL - 19
JO - Current Rheumatology Reports
JF - Current Rheumatology Reports
SN - 1523-3774
IS - 11
M1 - 71
ER -