Pulmonary hypertension, or PH, occurs when blood pressure within the lungs becomes abnormally elevated. It can be caused by a thickening of the pulmonary artery walls, heart failure, lung disease (such as interstitial lung disease), and clots within the lungs' blood vessels. It's common for ILD patients to also develop pulmonary hypertension.
PH should not be confused with ordinary hypertension — sometimes called systemic hypertension or high blood pressure. PH can occur in patients with or without ordinary hypertension.
PH in ILD is also a different disease than primary pulmonary hypertension — also called idiopathic pulmonary arterial hypertension — a rare condition in which PH develops in the absence of lung disease or any other known cause.
Symptoms of PH are similar to those of ILD. Patients with PH often complain of shortness of breath with physical activity that gradually worsens over time.
Patients may also experience the following symptoms:
In ILD patients, pulmonary hypertension is usually suspected when these symptoms are out of proportion to the severity of the patient's ILD.
In diagnosing PH, your doctor will start by conducting a thorough medical history and physical examination. This will include discussing your other medical problems, which may be associated with PH. A review of your medications and exposures is important, since certain diet pills and recreational drugs can increase the likelihood of having PH. The physical exam, which includes listening to your heart sounds and observing your neck veins, may also reveal findings suggestive of PH.
Your doctor may also conduct the following tests to make an accurate diagnosis:
Treatment for PH can be divided into two main categories: supportive therapy and PH-specific therapy. Supportive therapy is used to treat the underlying factors which may worsen PH, and to prevent complications. PH-specific therapies are designed to reduce the blood pressure within the lungs.
Several PH-specific therapies have recently been approved by the U.S. Food and Drug Administration (FDA) for use in patients with other forms of PH — specifically pulmonary arterial hypertension. The efficacy of these therapies in patients with PH and ILD remains uncertain. Using PH-specific therapy in ILD patients remains controversial, so you should discuss the benefits and risks with your provider.
Survival in patients with ILD and PH is worse than in ILD patients without PH. Several research studies are now looking at the role of PH treatment in improving the prognosis for these patients. If you are a good candidate for a lung transplant and have progressively worsening ILD and PH, your doctor may refer you to a specialist for a lung transplant evaluation
Reviewed by health care specialists at UCSF Medical Center.
This information is for educational purposes only and is not intended to replace the advice of your doctor or health care provider. We encourage you to discuss with your doctor any questions or concerns you may have.
Interstitial Lung Disease Program
400 Parnassus Ave., Fifth Floor
San Francisco, CA 94143
Phone: (415) 353-2577
Fax: (415) 353-2568