Pulmonary arterial hypertension (PAH) commonly occurs in chronic interstitial lung disease and idiopathic pulmonary fibrosis, conditions in which nonspecific interstitial pneumonia (NSIP) is also often found. For those suffering disorders of the lung, it is important to understand more about PAH and NSIP, including current means of diagnosis, as well as management and treatment options.
PAH refers to high blood pressure in the lungs and it’s a condition that worsens over time and can be potentially fatal since increased pressure in the lung arteries can strain the heart and cause heart failure. On the other hand, NSIP is a rare disease that affects the tiny air sacs, called alveoli, found within the lungs. The walls of the alveoli become swollen, as does the thin covering that protects the lungs, called the pleura. Two forms of NSIP exist, known as cellular and fibrotic. In the fibrotic form, there is additional scarring and the prognosis is poorer.
How can PAH and NSIP be related?
The frequency of occurrence and clinical features of pulmonary hypertension in patients with idiopathic NSIP are not fully understood due to a lack of reliable data. But it is believed that PH is most often diagnosed in interstitial lung diseases that involve connective tissue diseases whose underlying trigger is often nonspecific interstitial pneumonia.
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