Table 26

Recommendations for pulmonary arterial hypertension associated with connective tissue disease

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  • CTD: connective tissue disease; DLCO: diffusing capacity of the lung for carbon monoxide; IPAH: idiopathic pulmonary arterial hypertension; PAH: pulmonary arterial hypertension; RHC: right heart catheterization; SSc: systemic sclerosis.

  • aClass of recommendation.

  • bLevel of evidence.

  • cReference(s) supporting recommendations.