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Pulmonary arterial hypertension (PAH) is a rare lung condition. When you have PAH, small arteries in your lungs thicken and narrow, which makes it hard for your blood to flow. That puts strain on your heart as it works harder to pump blood through narrowed arteries. It’s a progressive disease, which means it gets worse over time. 

PAH can shorten your life span and have a big impact on your quality of life. The sooner it is diagnosed, the better your odds. Some studies suggest people from certain ethnic and minority groups with PAH don’t fare as well as white patients. Researchers are digging into the reasons for those differences. 

Causes of PAH

PAH can run in families and have a genetic cause. You also can develop PAH from exposure to certain toxic substances, including methamphetamines. Some weight loss drugs were taken off the market in the 1990s because they were linked to PAH. 

PAH also can crop up as a result of another illness, such as:

  • Liver disease
  • Heart disease
  • Connective tissue disorders such as scleroderma
  • HIV 
  • Sickle cell disease
  • Chronic obstructive pulmonary disease (COPD) 

The most common cause of PAH appears to differ among racial and ethnic groups. One study found that Black people with PAH were more likely to have connective tissue disease, and Hispanic people were more likely to have heart disease. The white participants in the study were more likely to have inherited PAH or to have exposure to toxic substances.

There were other demographic differences. The Black and Hispanic people with PAH in this study tended to be younger.

Differences in Risk of Getting PAH 

Some of the diseases that increase your chances of getting PAH are more common among certain populations. 

For instance, Black people have a higher risk of getting scleroderma, getting it at a younger age, and having a more aggressive version of the disease.

Sickle cell disease affects the Black population in the U.S. more than it does the Hispanic population. The condition is much less common among white people. 

Liver disease and HIV, two other risk factors for PAH, also are more common among the Black population in the U.S. 

Social and Economic Factors 

Social issues can affect how early your PAH is diagnosed and the way it’s treated.  Racial and ethnic minorities in the U.S. generally are more likely to experience problems such as: 

  • Lacking health insurance
  • Language barriers with health care providers 
  • Racial discrimination 
  • Not seeing the same doctor regularly 

Economics also play a role in your health. These issues can include: 

  • Low income
  • Health risks associated with your work 
  • Pollution
  • Substandard housing 
  • Poor nutrition 

Economic difficulties and individual experiences of dealing with racism, which can result in added stress, can also make you more vulnerable to illnesses such as high blood pressure and heart disease. Those are conditions that increase your odds of getting PAH.

People who deal with these economic and social hurdles often have more advanced cases of PAH when they finally do see specialists. Most current research on PAH doesn’t take these social and economic issues into account. 

Differences in Access to PAH Care

PAH can be difficult for doctors to pinpoint because the early symptoms are similar to many other conditions. It often takes 2 years or longer to find out exactly what’s wrong. To get the right diagnosis, you need to see specialists in lung disorders (pulmonologists) and heart disease (cardiologists) and take several specific tests. It can be difficult for patients from racial and ethnic minorities to get appointments with specialists. 

People with PAH fare best when they have a team coordinating all aspects of their care, including medication, lifestyle changes, and support for managing day-to-day life. Their health needs to be watched closely. An economic issue as simple as not having a car can make it difficult to get the best level of care.       

Studies and clinical trials of new treatments are key to advancing knowledge about PAH. But Black and Hispanic patients aren’t included in research  as often as white patients are. 

Registries of people with PAH are an important way for scientists to study the disease. Hispanic patients are underrepresented in all of the major registries. 

In clinical trials, which test new treatments, Black patients are underrepresented. 

Treatment guidelines for PAH end up being based on what works in a population that is whiter than the population of people with PAH. Also, Black and Hispanic patients end up having fewer opportunities to try out cutting-edge treatments offered in clinical trials. 

Differences in PAH Treatment 

Several medications and procedures can improve the lives of people with PAH. But there appear to be racial disparities in access to those treatments and how they work. 

In one study, Hispanic patients were less likely to receive the drugs that specifically target PAH. 

One type of drug used to treat PAH is endothelin receptor antagonists. ERAs, as they are called, help keep blood vessels from narrowing. But these drugs don’t work as well for Black patients as they do for those who are white.  Black people tend to have a higher level of endothelin – a protein that helps regulate your blood pressure – and produce even higher levels of this protein under stress. Researchers are looking into whether the current drugs don’t do enough to bring down endothelin levels in Black patients. 

More Data Is Needed 

Researchers need more data – especially on PAH among the Hispanic population – to know more about the role of race and ethnicity in PAH. 

According to some studies, PAH death rates are worse for Black patients – especially women – than they are for white patients.  A more recent, larger study found that race and ethnicity didn’t affect survival rates. But it was based on a registry of PAH patients, which might not represent the full range of people with PAH. 

Most research on PAH involves patients in the U.S, Europe, and Australia. Almost no data comes in from Central or South America, Africa, or Asia. 

Show Sources

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American Lung Association: “Learn about Pulmonary Arterial Hypertension,” “Pulmonary Arterial Hypertension Symptoms and Diagnosis,” “Treating and Managing Pulmonary Arterial Hypertension.” 

American Journal of Respiratory and Critical Care Medicine: “Health Disparities in Patients with Pulmonary Arterial Hypertension: A Blueprint for Action.”

National Organization for Rare Disorders: “Pulmonary Arterial Hypertension.” 

National Heart, Lung and Blood Institute: “Pulmonary Hypertension Causes and Risk Factors.” 

Pulmonary Circulation: “Racial and Ethnic Differences in Pulmonary Arterial Hypertension,” “Social determinants of health in pulmonary arterial hypertension patients in the United States: Clinician perspective and health policy implications.”

Advances in Pulmonary Hypertension: “Considering the Impacts of Health Disparities, Inequalities, and Inequities on Early Diagnosis of Pulmonary Arterial Hypertension.”

CDC: “Incidence of Sickle Cell Trait in the U.S.” 

European Respiratory Review: “Pulmonary arterial hypertension: tailoring treatment to risk in the current era.”

Chest: “What Rare Diseases Tell Us About Disparities in Disease Registries, Clinical Trials, and Treatment Algorithms,” “Race and Sex Differences in Response to Endothelin Receptor Antagonists for Pulmonary Arterial Hypertension,” “Pulmonary Hypertension Surveillance.” 

Cleveland Clinic: “Endothelin,” “Renin-Angiotensin-Aldosterone System (RAAS).”

Journal of Heart and Lung Transplantation: “Impact of race on survival in pulmonary arterial hypertension: Results from the REVEAL registry.”

Cureus: “Pulmonary Arterial Hypertension in Hispanics.”