PH and Lupus

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What do I need to know about lupus-associated PH?

First of all, it is essential to know that PH and lupus are two separate conditions and that PH does not cause lupus. However, some lupus patients develop PH. As was discussed in the introduction, there are a number of reasons why blood pressure in the lung may become elevated — almost all of which can occur in lupus. For this reason, it is very important that patients with lupus and suspected PH be looked at very carefully to understand exactly what might be contributing to PH.

Lupus-associated PH may be due to more than just one problem. Other causes of PH include left-heart dysfunction, vasculitis (an inflammation or irrita- tion of small blood vessels in the lung), pulmonary embolism (blood clots in the lungs) or pulmonary parenchymal disease (scarring or irritation of the area surrounding the air sacs and blood vessels in the lungs). Each of these situations would be treated differently. Since this is much more complex than PAH alone, receiving care from a physician who is experienced in the management of lupus and PAH is important.

One of the most serious kinds of PH is PAH. This remains a relatively uncommon complication of lupus, but because PAH can be very problematic, it is important to find and treat this as soon as possible. Current estimates suggest that somewhere between 0.5 and 9 percent of patients with lupus may have PAH.

Lupus remains a very complex process that can affect one or many organs. In particular, lupus can cause kidney, heart or lung dysfunction in some people, which can lead to shortness of breath, fatigue or fluid retention — all of which can also occur in PAH. These additional factors particular to lupus can complicate management of PAH and may require additional testing to ensure the best treat ment of lupus-associated PAH.

Why do PH and lupus seem to occassionally occur together?

This is another question that medical science is searching to answer. Medical researchers have not yet been able to say for sure how lupus leads to the develop- ment of PAH in some patients. 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. Lupus remains a complicated disease that can cause many problems that weaken blood vessels, and these, too, might play into the development of PAH.

Who is most susceptible to a lupus-associated PAH?

Lupus-associated PAH occurs more frequently in females and in a slightly younger age group than idiopathic PAH (IPAH) or scleroderma-associated PAH. There also appear to be racial differences in lupus-associated PAH with increased frequency of non-Caucasian patients compared to IPAH or scleroderma-associated PAH. There are no blood tests able to accurately predict if a lupus patient
has PAH or might develop it.

Should a lupus patient be tested for PH?

Lupus patients know that medical supervision is critical to their best possible long-term health and that any change in their condition should be brought to the attention of their doctors. Changes that may have to do with PH might include difficulty when attempting physical activity; swelling of the feet, ankles, legs or abdomen; increased shortness of breath; worsening fatigue; chest discomfort or pain; light-headedness or fainting. These changes should be communicated to your doctor promptly.

How is testing for PH carried out?

A visit to your doctor and a thorough exam is a good first step for evaluation of potential PH. An echocardiogram is a very useful test that looks at the size and functions of the heart among other things and can suggest whether PH may exist. Unfortunately, it alone is not accu- rate enough to make the diagnosis of lupus-associated PAH. A test known as right-heart catheterization remains the most accurate way of diagnosing lupus- associated PAH. Additional testing is also typically done to see how much exercise you can do and to check lung function or x-rays to be sure that additional problems in the lungs are not present.

Can PH be treated the same way in lupus patients as in those without lupus?

Through the years, a variety of medications have been shown to slow the damage and relieve the symptoms caused by PAH. These medications are very complex, and choosing the correct initial treatment requires the knowledge and expertise of a physician who specializes in PAH. Professionals at PH centers can help identify PH and choose the correct type of treatment.

In addition to medications approved for the treatment of PAH, patients may benefit from simple basic measures including attention to fluid balance and therapies that improve the efficiency of the heart. These may include supplemen- tal oxygen or the medication digoxin, as well as anticoagulants (blood thinners).

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. 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.

Does having lupus make PH worse?

The answer is yes and no. In general, patients who have lupus-associated PAH tend to respond to therapy similarly to IPAH patients who do not have lupus. As was noted above, however, patients with lupus may have other problems such as left-heart dysfunction, blood clots in the lungs, inflammation of the lungs or its blood vessels. All of these could make PH worse for that person.

Michael J. Krowka, MD
Mayo Clinic, Rochester, Minn.

Michael A. Mathier, MD, FACC;
University of Pittsburgh School of Medicine, Pittsburgh, Pa.

Robert Schilz, DO, PhD,
Case Western Reserve University School of Medicine,
Cleveland, Ohio

This page last updated December 2013

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