CTD-PAH: Adjusting Treatment Based on Patient Factors

Patient factors that influence the management of connective tissue disease—associated pulmonary arterial hypertension.


Hilary M. DuBrock, MD: Treating connective tissue disease—associated pulmonary arterial hypertension [PAH] can be challenging because these patients tend to respond less well to PAH therapy and can progress despite maximum medical therapy. Additionally, there can often be occult heart failure with preserved ejection fraction in patients with connective tissue disease–associated pulmonary arterial hypertension, or occult pulmonary venous involvement, which can impact treatment response.

My approach to treating patients with connective tissue disease—associated PAH tends to be to perform more serial and regular right heart catheterizations, particularly with exercise, to ensure that we can detect occult heart failure with preserved ejection fraction as a cause of a patient’s clinical worsening over time and to make sure that I use the right treatment approach as I add any additional PAH-targeted therapy.

Although treating connective tissue disease—associated PAH—particularly scleroderma-associated pulmonary arterial hypertension—can be challenging, on the other end of the spectrum, patients with connective tissue disease–associated PAH also associated with lupus can be rewarding to treat. Sometimes, the pulmonary hypertension can resolve with immunosuppression. I’ve had patients with lupus-associated pulmonary arterial hypertension on triplet therapy for pulmonary arterial hypertension who are now off of all PAH therapy. This is a rare but rewarding experience in treating patients with pulmonary arterial hypertension.

Charles D. Burger, MD: The guidelines struggle a little bit when patients have very mild disease. Let’s say they’re able to do most things in life but with heavy exertion, they’re short-winded. Their examination and their biomarkers, such as BNP [B-type natriuretic peptide] and 6-minute walk, look pretty good. The echocardiogram shows only mild stress on the heart. It could be that a lot of those patients, or a certain percentage of those patients, would do OK on 1 drug. It’s not known for sure.

But in subpopulations such as PAH associated with connective tissue disease, where patients tend to be more severely affected, have the additional challenge of connective tissue disease, and have a more challenging prognosis, my bias—based on my experience and my reading and interpretation of the published literature—is that this is a group where you would be inclined to use upfront combination therapy and not approach it with just 1 drug, barring any extenuating circumstance.

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