The term connective tissue disease (CTD) refers to a group of autoimmune disorders
that are classified among the systemic rheumatic diseases [
[1]
]. Pulmonary arterial hypertension (PAH) is a common and severe complication of such
CTDs as systemic sclerosis (SSc), mixed CTD, systemic lupus erythematosus and, to
a lesser extent, polymyositis-dermatomyositis, rheumatoid arthritis, antisynthetase
syndrome and others [
2
,
3
,
4
]. CTD-associated PAH (CTD-PAH) is the second most prevalent type of PAH after idiopathic
PAH (IPAH) in Western world, affecting 0.5–15% of patients with CTDs and is one of
the leading causes of mortality in patients suffering from SSc and mixed CTD [
5
,
6
,
7
]. PAH is defined as elevated mean pulmonary arterial pressure (mPAP) ≥25 mm Hg at rest with presence of pre-capillary pulmonary hypertension (PH), defined by
pulmonary artery wedge pressure (PAWP) ≤15 mm Hg and a pulmonary vascular resistance (PVR) >3 Wood units in the absence of other causes of precapillary PH [
[2]
].To read this article in full you will need to make a payment
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References
- Defining unclassifiable connective tissue diseases: incomplete, undifferentiated, or both?.J Rheumatol. 2005; 32: 213-215
- 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.Eur Heart J. 2016; 37: 67-119https://doi.org/10.1093/eurheartj/ehv317
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- Pulmonary arterial hypertension in France: results from a national registry.Am J Respir Crit Care Med. 2006; 173: 1023-1030https://doi.org/10.1164/rccm.200510-1668OC
- Recommendations for screening and detection of connective tissue disease-associated pulmonary arterial hypertension.Arthritis Rheum. 2013; 65: 3194-3201https://doi.org/10.1002/art.38172
- The REVEAL registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension.Chest. 2012; 141: 354-362https://doi.org/10.1378/chest.11-0676
- Predicting survival in pulmonary arterial hypertension in the UK.Eur Respir J. 2012; 40: 604-611https://doi.org/10.1183/09031936.00196611
- Pulmonary hypertension in adults with congenital heart disease: first data from Latvian PAH registry.Eur J Intern Med. 2016; 36: e20-e21https://doi.org/10.1016/j.ejim.2016.09.002
Article info
Publication history
Published online: January 17, 2017
Accepted:
January 11,
2017
Received in revised form:
January 4,
2017
Received:
January 3,
2017
Identification
Copyright
© 2017 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
