Pulmonary Hypertension and Sarcoidosis

Pulmonary hypertension (PH) is a rare but life-threatening lung disorder that damages the vessels responsible for transporting blood from the heart to the lungs, known as the pulmonary arteries. The disease is characterized by high blood pressure due to narrowed and thickened pulmonary arteries. Since the right heart ventricle needs to work harder to properly pump blood, the heart becomes enlarged and weakened, which can result in right heart failure.

There is currently no cure for pulmonary hypertension, and if not treated it can lead to death. However, there are treatments to help cope with the symptoms. Many factors can impact the prognosis of the disease, including other medical conditions. Pulmonary hypertension can be both caused by, or cause, other diseases.

Among them is sarcoidosis, a multisystem inflammatory disease characterized by noncaseating granulomas, particularly in the lungs and intrathoracic lymph nodes.

Development and Causes of Pulmonary Hypertension and Sarcoidosis

The inflammatory disease sarcoidosis has an unknown etiology and affects about 11 people in every 100,000 in whites but about 34 cases per 100,000 population in African Americans. The disease is found through asymptomatic symptoms such as chest imaging; systemic and pulmonary complaints such as fever, weight loss,  dyspnea on exertion, cough and chest pain; and Löfgren syndrome.

According to the study “Sarcoidosis-associated pulmonary hypertension,” which analyzed the clinical characteristics of patients with sarcoid-associated pulmonary hypertension (SAPH), pitfalls in the diagnosis, and potential therapies, pulmonary hypertension is a serious complication of sarcoidosis.

The combination of the two diseases can occur in any patient, but it is more common among patients in an advanced stage. Restrictive lung physiology, hypoxemia, advanced Scadding chest X-ray stage, and low carbon monoxide diffusion capacity are important risk factors that impact the development of pulmonary hypertension in patients with sarcoidosis. In order to diagnose the disease, an echocardiogram serves as an initial screening tool, but right heart catheterization is necessary to confirm it. Patients who experience persistent dyspnea are advised to be screened for the disease.

Pulmonary Hypertension and Sarcoidosis Prevalence and Management

Despite the fact that sarcoidosis has an overall mortality between 1 and 5 percent, sarcoidosis-associated pulmonary hypertension is a life-threatening complication that increases both morbidity and mortality significantly, as explained in the “Pulmonary hypertension caused by sarcoidosis.” There is a poor prognosis for patients suffering from the two conditions. However, there are treatments that can ease the symptoms, improve quality of life, and life expectancy.

In the case of pulmonary hypertension and sarcoidosis, the most common treatments include pulmonary vasodilators, such as endothelin antagonists, and anti-inflammotary agents, which can help lower pulmonary arterial pressures, improve dyspnea and functionality, and enhance overall quality of life. Due to the severity of the disease, patients can also be added to the transplantation waiting list to receive a new lung or both lungs.