We discussed a young male with chief complaint of dyspnea on exertion that was progressive. Other symptoms included GERD, skin tightening on face and fingers, Raynaud’s in digits who was found to have hypoxic respiratory failure and severe RV enlargement. Right heart cath showed mean PA pressure of 32 mm Hg with wedge pressure 3mmHg and RVSP 52mmHg, CI 4.15L/min/m2. He was diagnosed with PAH (group 1 PH) likely due to underlying ILD from connective tissue disease, specifically Limited Cutaneous Systemic Sclerosis (CREST).

PH is defined as mean PA pressure (PAP) of >20mmHg at rest. Here is the 5 groups of PH. Remember PAH is a specific subgroup of PH. Group 1, 3 and 4 require mean PAP >20mmHg; Wedge<15mmHg and PVR>3 WU. Group 2 and 5 require mean PAP >20mmHg, wedge>15mmHg and PVR <3Wu.

Remember ANA is positive in 70% of systemic sclerosis but other antibodies are associated with more specific complications of systemic sclerosis. Here is a table to summarize.