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Autoimmune Disease-Associated Pulmonary Arterial Hypertension Versus Idiopathic Pulmonary Arterial Hypertension: Which Subset of Patients Receives More Combination Therapy

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Conference: 2014 International PHA Conference and Scientific Sessions

Release Date: 06.22.2014

Presentation Type: Abstracts

File Download: 2014 Conference Abstract - Maria Hymon

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Pulmonary arterial hypertension (PAH) is a rare disease with significant associated morbidity and mortality. Almost half of all PAH is idiopathic in origin with a nearly equal amount having an associated etiology. Of the associated etiologies, approximately half are secondary to connective tissue diseases.

Background: Pulmonary arterial hypertension (PAH) is a rare disease with significant associated morbidity and mortality. Almost half of all PAH is idiopathic in origin with a nearly equal amount having an associated etiology. Of the associated etiologies, approximately half are secondary to connective tissue diseases. The 3 year mortality rate in idiopathic PAH is estimated to be 41% whereas the mortality rate in autoimmune disease-associated PAH is estimated to be much higher. The difference in pathophysiology and mortality between these two groups lends credence to the question of combination therapy and differences in clinical practice for these two groups.

Methods: Retrospective review of patient data in a community PAH clinic with categorization of purported PAH etiology and current treatment regimen.

Results: Study cohort consisted of 73 total patients all of whom were on phosphodiesterase 5 inhibitors (PDE5), endothelin receptor antagonists (ERA), prostacyclins, or a combination of 2 or more agents. 72% of patients were female. 60% of patients were classified as idiopathic PAH whereas 10% were classified as autoimmune disease-associated PAH. Of these two groups, 47% of idiopathic PAH patients were on combination therapy versus 25% of the autoimmune disease-associated patients. Of the combination therapies, 19% consisted of an ERA plus a PDE5 inhibitor, and17% consisted of an ERA plus a prostacyclin. 

Conclusion: Little data exists to define the optimal treatment regimen for PAH in different populations. Retrospective data review showed that within our patient cohort, the idiopathic PAH patients were more likely to be on combination therapy than the autoimmune disease-associated PAH patients with nearly equal percentages on combination therapy with an ERA plus a PDE5 inhibitor versus an ERA and a prostacyclin. Multiple potential etiologies exist for the differences in rates of combination therapy use such as differences in where patients are in their disease process at the time of recognition and initial intervention. The different rates of combination therapy use is worth further future exploration as it would be important to better understand the reason for this dichotomy and to rationalize current practice or to accentuate the need for alterations in current practice despite the paucity of data available to standardize practice.