11th January 2024

Pulmonary arterial hypertension

This is a frequent late-stage complication of many ILDs, in particular IPF, sarcoidosis and CTD-ILD.
As fibrosis progresses, affected parts of the lung become hypoxic. This leads to reactive vasoconstriction and redistribution of the blood to better-ventilated parts of the lung. These processes, in turn, lead to rise in intravascular pressure, wall stress and further release of inflammatory and proliferative cytokines. It also puts stress on the right heart, which has to pump against the high pressures, eventually leading to right heart failure.
ILD-related PAH is associated with reduced exercise capacity, poorer quality of life, and shorter life span.
It can be difficult to diagnose, as symptoms are similar to those of ILD. It should be suspected in any patient showing significant clinical deterioration, especially when it seems to be out of proportion to their lung function tests, and in any patient showing signs/symptoms of heart failure.
Echocardiogram is usually the first investigation as it is a great non-invasive test to screen for the condition. Right heart catheterisation (RHC) remains the gold standard; mean pulmonary artery pressure more than 20 mm Hg is diagnostic.
Treatment options include supportive management, including supplemental oxygen, and heart failure therapy. There are currently no medical therapies approved for ILD-PAH (note that this is different from the primary pulmonary hypertension, which has multiple treatment options).

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