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  • Writer's pictureSarah Schafer, MD

Pulmonary hypertension

Updated: Nov 28, 2023

What is pulmonary hypertension?

Pulmonary hypertension (PH) means high blood pressure in the lungs. There are multiple types of pulmonary hypertension. In Sjogren’s, this usually comes in the form of pulmonary arterial hypertension (PAH).

PAH is serious; it may lead to heart failure and early mortality. It is also seen in other autoimmune rheumatic diseases (AIRDs* ), especially systemic sclerosis.

Early detection, diagnosis, and treatment are critical to improving outcomes.

* AIRDs are often called connective tissue diseases (CTDs). See the Glossary to learn more about these terms.

How common is pulmonary hypertension in Sjogren’s?

Data from tertiary care centers found signs of pulmonary hypertension on screening echocardiography in 12.5-23.4 % of Sjogren’s patients. This study excluded patients with other AIRDs (241). 23.4% is likely to be higher than the true prevalence because tertiary care centers see patients who are sicker on average. Whether the true prevalence is 5%, 10%, or 25%, PAH should be on the radar of every rheumatologist.

Likewise, pulmonologists should consider the possibility of undiagnosed Sjogren’s in patients with idiopathic PAH (unknown cause). One study of Sjogren’s-PAH patients found that 41.4% were diagnosed first with PAH (246). Sjogren's can be challenging to diagnose in patients that present with PAH because 50% have no obvious sicca and are more likely to be ANA negative (247).

Are there risk factors for PAH?

Yes, although anyone with Sjogren's may develop PAH. Sjogren’s patients who develop PAH are more likely to have Raynaud’s phenomenon, high- titer rheumatoid factor, liver injury, and pericardial effusion (fluid accumulation around the heart) (246).

PAH impacts both SSA positive and SSA negative patients.

What are the symptoms of PH/ PAH?

Shortness of breath on exertion (exercise intolerance) is the most common presenting symptom. In more advanced disease, patients may have difficulty performing activities of daily living. Other symptoms include cough, heart palpitations, rapid heart rate, fainting, swelling of the ankles and legs (edema), chest pain, fainting, and fatigue.

If you are experiencing symptoms indicative of PAH or if you have had a cardiac echo with an elevated pulmonary pressure of 25 or greater you should be further evaluated for PAH. PAH is not measured by pulmonary function tests.

What if my doctor does not take my symptoms seriously?

Until recently, PAH was considered a rare manifestation of Sjogren’s. Rheumatologists are often unaware of the high rates of PAH and other types of lung disease in Sjogren’s patients. Patients often need to advocate for a thorough pulmonary evaluation when their symptoms are downplayed or incorrectly attributed to being unfit, overweight, or depressed. Tips for sharing educational material with your doctors.

See the blog post, What you should know about lung disease, to learn how to use the Pulmonary Clinical Practice Guidelines and the journal article from CHEST to advocate for an evaluation.

How is pulmonary hypertension diagnosed?

The first test done is usually an echocardiogram. The diagnosis is usually confirmed by performing a right heart catheterization. Pulmonary function tests and chest X-rays are usually normal. Your doctor may hear lung "crackles" when listening to your lungs with a stethoscope.

If you are experiencing symptoms indicative of PAH or if you have had a cardiac echo with an elevated pulmonary pressure of 25 or greater you should be further evaluated for PAH. PAH is not measured by pulmonary function tests.

"Pulmonary hypertension should move to the top of the possible causes

for people with shortness of breath, swelling of the legs, chest pain and

fainting and normal pulmonary function tests."

In Sjogren’s, PAH can develop on its own or it can be caused by ILD and pulmonary fibrosis (scarring). A full lung evaluation according to the Pulmonary Clinical Practice Guidelines is indicated for all Sjogren’s patients with lung disease symptoms. A high-resolution CT (HRCT) should be used for symptomatic patients. A chest X-ray will not detect most Sjogren’s lung disease.

See What you should know about lung disease for details and links.

How is pulmonary hypertension treated?

Treatment approaches vary, based on the type of pulmonary hypertension and underlying cause(s). It is important for newly diagnosed patients to find a PH specialist who can accurately find what is causing their PH and develop a treatment plan that is right for their specific type of PH as soon as possible. Learn more about pulmonary hypertension:

Pulmonary Hypertension Association (PHA) The PHA's Sjogren’s and PH brochure. This has good info, other than stating that PH is rare in Sjogren's.

Many thanks to Christine Shields, RN, for her insightful comments and resources.

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