There is a paucity of research and a lack of necessary guidance for proper characterization of pulmonary
hypertension presenting in patients with connective tissue disease and interstitial lung disease, specifically related to
differentiating between WHO group 1 pulmonary arterial hypertension and WHO group 3 pulmonary hypertension due to
lung disease and/or hypoxia. Without a mechanism for proper differentiation of the type of pulmonary hypertension that
exists, appropriate management of this subset of individuals is particularly challenging. This review presents the pertinent
research that exist regarding the intricacies of this disease state with the goal of providing improved guidance as to who
may benefit from screening, diagnosis, treatment in a facility with expertise in the field, and referral for lung transplant.
Keywords: Pulmonary hypertension, pulmonary arterial hypertension, interstitial lung disease, connective tissue disease,
parenchymal lung disease, lung transplant.
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