Pulmonary arterial hypertension (PAH) is a rare lung disease and a form of pulmonary hypertension (PH) that damages the arteries and causes high blood pressure in the lungs. Here are some key points about PAH:
- Prevalence: PAH affects approximately 2 to 8 people per million worldwide, with about 500 to 1,000 new cases Show Full Answer
Pulmonary arterial hypertension (PAH) is a rare lung disease and a form of pulmonary hypertension (PH) that damages the arteries and causes high blood pressure in the lungs. Here are some key points about PAH:
- Prevalence: PAH affects approximately 2 to 8 people per million worldwide, with about 500 to 1,000 new cases diagnosed each year in the United States.
- Types:
- Heritable PAH (hPAH): Linked to family history and genetic mutations, particularly on the BMPR2 gene.
- Idiopathic PAH (iPAH): Has no clear cause.
- Associated PAH (aPAH): Linked to other medical conditions like connective tissue disease, congenital heart disease, liver disease, kidney disease, and HIV.
- Symptoms: Vary from person to person and depend on the stage of the disease and the individual's age.
- Diagnosis: Often delayed, taking up to two or more years due to nonspecific symptoms and lack of training among doctors to recognize PAH signs.
- Risk Factors:
- Gender: Women are almost twice as likely to develop PAH as men, though this ratio decreases with age.
- Genetics: 15% to 20% of PAH cases are heritable.
- Race and Ethnicity: Differences in types of PAH across racial and ethnic groups have been observed, though more research is needed.
- Treatment: Includes medications to reduce symptoms, and in advanced cases, lung transplants may be advised. Regular evaluations every three to six months are recommended to monitor disease progression.
For more detailed information, you can refer to the resources provided by myPHteam.
July 14, 2024