How do you approach management of a patient with idiopathic PAH and +ANA, but no other current symptoms/signs of active rheumatologic disease?

(1) Of note, abnormal nail fold capillaroscopy is associated with PAH in several systemic immune diseases: This is famously true for systemic sclerosis, but there are also publications reporting this association for other diseases, notably those in the “lupus family“ like SLE and dermatomyositis.

As...

First, I'd collect specific antibodies we have for diseases that have PAH associations like SLE, Sjogrens, systemic sclerosis, and MCTD to make sure not to miss a diagnosis in addition to checking inflammatory markers and UA.

If all ANA sub-serology is negative: it's important to look at BMI, OSA, etc.