Sarcoidosis is an inflammatory disease that can affect many different organs in the body. The exact cause is a mystery to doctors and researchers.  

Sarcoidosis often causes problems in the lungs, which leads to shortness of breath with activity due to: 

• Lung tissues (scarring/fibrosis or inflammation)

• Airways (similar to asthma)

• Blood vessels (elevated pressure or PH)

In sarcoidosis-associated PH, increased pressure in the lungs can weaken the right side of the heart and ultimately cause it to fail.  

Sarcoidosis-related PH can happen for several reasons. Fibrosis, inflammation and airflow obstruction all can cause some degree of PH, since they make it harder for the lungs to transfer oxygen. Furthermore, the fibrosis itself can distort and destroy the lungs’ blood vessels, elevating pressures.  

Some patients have enlarged lymph nodes, which put pressure on lung vessels and cause PH. In others — when there is little or no fibrosis, inflammation or airflow obstruction — PH is thought to be the result of sarcoidosis directly affecting pulmonary vessels. 

Diagnosing PH 

It’s important to report changes in symptoms, such as unexplained shortness of breath; increased fatigue; swelling of the ankles, legs or abdomen; chest discomfort or pain; and light-headedness or fainting. All can be signs of sarcoidosis-related PH. People with sarcoidosis often have exercise limitations.  

Pulmonary complications are the leading cause of death in sarcoidosis patients, so regular follow-up visits with your health care team are important. While many sarcoidosis patients go on to lead long lives without much trouble from their disease, sarcoidosis-related PH is a serious and complex condition that requires further evaluation.  

Declining diffusion capacity is known to be associated with PH development among people with sarcoidosis. In addition, many patients whose oxygen levels fall during a six-minute walk test also have underlying SAPH.  

Treatment options  

All people with sarcoidosis-related PH should have therapies to optimize their sarcoidosis-specific treatment, including immunosuppressive therapies and bronchodilators. In addition, patients should pay attention to optimal fluid balance with diuretics (water pills) and a low-sodium diet. Patients should be evaluated for low oxygen levels because oxygen levels can fall when exercising and sleeping.  

Exercise is of utmost importance. Cardiopulmonary rehabilitation has been shown to improve symptoms and exercise capacity for people with various forms of PH.  

There is limited information about the effectiveness of PH-specific medications for people with sarcoidosis. Several studies that could provide more information are underway. The decision whether to use PH-specific medications can be complicated and requires careful follow up by a PH specialist.  

Download or order a brochure

Information on PH and Sarcoidosis is one of a series of PH& brochures available to download as a PDF or to order as a printed brochure. 

PH & Sarcoidosis