Lupus and PHPulmonary hypertension (PH) and the more serious pulmonary arterial hypertension (PAH), involves high blood pressure in the lungs.  PAH has a number of causes that can create a chronic and life-changing disease that may lead to right heart failure if untreated.

Lupus is an autoimmune disease that generates chronic inflammation. Lupus may cause disease of the nervous system, joints, kidneys, lungs and skin.  Early symptoms include red, scaly rashes on the cheeks and nose and may spread to the extremities.  The cause of lupus is not known, however it has been observed that heredity, viruses, UV light and drugs may contribute to the disease.

PH and lupus are two separate diseases, and it is important to know that pulmonary hypertension does not generate or cause lupus.  Nevertheless, some lupus patients may develop PH over time.  Lupus may generate high blood pressure in the lungs, and for that reason individuals with lupus and suspected PH should be examined thoroughly to understand what may be contributing to PH.  The Pulmonary Hypertension Association has recently posted a guideline on these two diseases.  This guideline may be of interest to patients who have lupus.

Lupus-associated pulmonary hypertension can be due to many issues.  PH symptoms may involve left-heart dysfunction, right-heart dysfunction, inflammation of the small blood vessels in the lung, pulmonary embolism (blood clots) or irritation of the area around air sacs.  Each of these symptoms require different treatments making lupus-associated PH more complex than PH on its own.  It is important that patients seek medical attention from a physician who is experienced with lupus-associated PH.

Lupus is a complicated disease that may affect many organs and generate symptoms such as fatigue, edema (fluid retention) and/or shortness of breath. When the symptoms overlap with PH and in worse cases PAH, management is difficult and can require additional testing to determine the best possible treatment.

To date, medical researchers have not been able to determine how lupus can lead to the development of PAH.  It is not known whether lupus can directly generate PH or whether it is a trigger in susceptible patients.  Lupus is known to weaken blood vessels which may contribute to PAH.  Often, lupus patients are administered steroids and steroids weaken blood vessels even more.

Chances For Developing Lupus-Associated PAH

Lupus-associated PAH occurs more often in females and in a somewhat younger group than idiopathic (unknown causes) PAH or scleroderma-associated PAH.  Scleroderma is another autoimmune disease that causes skin and possibly other organs to become thickened and hard.  There is an increase in probability of developing Scleroderma among non-Caucasian patient demographics.  There are no blood tests available that can accurately determine if a patent has or might develop lupus-associated PAH, making it important that report any change in condition to healthcare providers.  Changes can include swelling of the extremities or abdomen, an increase in difficulty breathing, increased fatigue, chest pain, and fainting.

Is there a test for PH and Lupus-Associated PAH?

Fortunately, there are diagnostic tests for pulmonary hypertension and lupus-associated PAH.  Healthcare providers can perform an echocardiogram to determine the size and functional capacity of a patient’s heart but the test is not sufficient to determine lupus-associated PAH.  A right-heart catheterization is a reliable way for doctors to diagnose lupus-associated PAH.  Additional tests such as exercise tests and X-rays can also check lung function and help determine if other health issues exist.

Treatment of Lupus-associated PH 

There are several drugs available that can slow down progression of lupus-associated PH and PAH as well as relieve symptoms.  These drugs are complicated and require a physician who specializes in PAH.  Chances are, patients will usually attend a PH center where specialists in PAH will determine the best form of treatment.  Additional therapies may include supplemental oxygen or digoxin (blood thinner).  Some patients with lupus-associated HP can benefit from immunosuppressive drugs which have the ability to decrease the amount of inflammation in the body.

Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.