Pulmonary hypertension (PH) is a disease that causes high blood pressure in the lungs due to narrowing in the pulmonary arteries. It is a rare but life-threatening condition that develops when narrowed vessels, which are responsible for transporting the blood from the heart to the lungs, force the heart to work harder to pump blood. In that case, the heart can become large and weak, resulting in right heart failure.

Identifying the reasons for the development of PH is one of the first and most important steps to properly treating the condition. There are cases in which the causes are not determined, known as idiopathic pulmonary hypertension. But numerous factors can trigger high blood pressure. PH can be caused by an underlying disease such as lupus, an autoimmune disease that occurs when the immune system attacks its tissues, resulting inflammation, swelling, pain, and damage.

Development of Pulmonary Hypertension in Patients with Lupus

Pulmonary hypertension and lupus are two very different conditions. Though pulmonary hypertension does not cause lupus, the autoimmune diseases can cause PH – though the coexistence of the diseases is rare. There are reasons for high blood pressure and almost of them can be found in those who have lupus, as explained in the guidelines issued by the Pulmonary Hypertension Association (PHA).

Lupus is a complex condition that damages one or more organs, particularly the kidney, heart, and lungs. The most common signs associated with the disease are shortness of breath, fatigue and fluid retention – symptoms that can also be experienced by patients with PH. The development of lupus-associated PH is not completely understood and may be caused by more than just one problem. According to the report: “It is not yet known, for example, whether lupus itself can directly cause PH, or whether lupus is simply a trigger for the development of PAH in susceptible individuals.”

Pulmonary Hypertension and Lupus Diagnosis and Prevalence

Pulmonary hypertension and lupus is more common among Caucasian and female patients younger than patients typically with PH. Early diagnosis if important because the effects can PH can worsen the symptoms of lupus.

There are no blood tests that can accurately detect PH in a lupus patient, but there are precautions that can be taken. Medical supervision is the best method to prevent it. Reasons to seek medical advice include: swelling of the feet, ankles, legs and abdomen, worsening in the difficulties to breath, chest pain, light-headedness or fainting. A physician may then request an echocardiogram to analyze the size and function of the heart. A right-heart catheterization can complete the diagnosis.

Treatment of Pulmonary Hypertension in Patients with Lupus

There is currently no cure for either pulmonary hypertension or lupus, but there are treatments that can help ease symptoms. According to the Pulmonary Hypertension Association, pulmonary specialists can help determine the most adequate treatment for lupus-associated pulmonary hypertension. Patients can be treated with medication approved by the U.S. Food and Drug Administration (FDA). The treatments for PH can be complemented with simple basic measures such as attention to fluid balance, and therapies that improve the efficiency of the heart like supplemental oxygen, digoxin, or anticoagulants.

“Once a doctor and patient have been able to establish an effective medical approach, many patients show significant improvement, often in conjunction with doctor-prescribed cardiopulmonary rehabilitation training (CPRT). A few patients with lupus-associated PH may also benefit from a group of drugs called immunosuppressive medications,” according to PHA guidelines. “These medications treat irritation or inflammation in the body, which can occur in lupus and occasionally can cause additional damage in the blood vessels of the lungs. It is difficult to know in all cases which patient may benefit from these kinds of medications,” is stated in the report.