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Pulmonary arterial hypertension associated with connective tissue disease: Insights from Latvian PAH registry

Published:January 17, 2017DOI:https://doi.org/10.1016/j.ejim.2017.01.010
      The term connective tissue disease (CTD) refers to a group of autoimmune disorders that are classified among the systemic rheumatic diseases [
      • Doria A.
      • Mosca M.
      • Gambari P.F.
      • Bombardieri S.
      Defining unclassifiable connective tissue diseases: incomplete, undifferentiated, or both?.
      ]. 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 [
      • Galiè N.
      • Humbert M.
      • Vachiery J.-L.
      • Gibbs S.
      • Lang I.
      • Torbicki A.
      • et al.
      2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.
      ,
      • Hervier B.
      • Meyer A.
      • Dieval C.
      • Uzunhan Y.
      • Devilliers H.
      • Launay D.
      • et al.
      Pulmonary hypertension in antisynthetase syndrome: prevalence, aetiology and survival.
      ,
      • Simonneau G.
      • Robbins I.M.
      • Beghetti M.
      • Channick R.N.
      • Delcroix M.
      • Denton C.P.
      • et al.
      Updated clinical classification of pulmonary hypertension.
      ]. 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 [
      • Badesch D.B.
      • Raskob G.E.
      • Elliott C.G.
      • Krichman A.M.
      • Farber H.W.
      • Frost A.E.
      • et al.
      Pulmonary arterial hypertension: baseline characteristics from the REVEAL registry.
      ,
      • Humbert M.
      • Sitbon O.
      • Chaouat A.
      • Bertocchi M.
      • Habib G.
      • Gressin V.
      • et al.
      Pulmonary arterial hypertension in France: results from a national registry.
      ,
      • Khanna D.
      • Gladue H.
      • Channick R.
      • Chung L.
      • Distler O.
      • Furst D.E.
      • et al.
      Recommendations for screening and detection of connective tissue disease-associated pulmonary arterial hypertension.
      ]. 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 [
      • Galiè N.
      • Humbert M.
      • Vachiery J.-L.
      • Gibbs S.
      • Lang I.
      • Torbicki A.
      • et al.
      2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.
      ].
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