15 Questions - “Take a Deep Breath” - Pulmonary Issues and Lupus

Inflammation caused by lupus may affect the lungs in many ways, and can involve the membrane lining of the lungs, the lungs themselves, the blood vessels within the lungs, and the diaphragm. This month, Dr. Patricia Eshagian answered your questions about pulmonary issues and lupus.

1. What are some of the symptoms of potential pulmonary problems with lupus? What clinical signs would a doctor detect? Apache Junction, AZ and Virginia Beach, VA

This is a great question to start with. Lupus can affect the lungs in many different ways. There may be pulmonary manifestations in the lung itself, the lining around the lung (or pleura), the blood vessels of the lungs, and/or the diaphragm or major breathing muscle. I’ll review some of the potential pulmonary problems here and what symptoms the process may cause and what your doctor may look for.

One common pulmonary problem includes "Pleuritic chest pain," which usually manifests as pain when breathing in. This may be caused by musculoskeletal pain or pain from inflammation around the costochondral joints. Your doctor has to carefully listen to what kind of your symptoms you are experiencing and in this case may be able to elicit pain by palpating the painful areas of the chest area.

Pleuritic chest pain may also be caused by inflammation around the lining of the lung or pleura. This can be a difficult diagnosis to make but often times your doctor may look for fluid around the lining of the lung on an x-ray or CAT scan to help make this diagnosis.

Certainly additional pulmonary problems in lupus include infection in the lung, especially in those who take medications that lower the immune system. In this case the clinical signs of infection usually include cough, fever, and shortness of breath or fatigue. Your doctor may need to get an x-ray to help make a diagnosis of infection or may need to check a sputum sample.

Lupus can also cause what’s called interstitial lung disease (ILD) which is a manifestation of inflammation in the lungs. The symptoms of ILD may include gradual increasing shortness of breath and a decrease in ones exercise tolerance. Your doctor will need to examine the lungs carefully and may hear abnormal lung sounds that help tip off this diagnosis. In this case an x-ray or CAT scan will be helpful in making the diagnosis, and your doctor may want to get breathing studies or pulmonary function tests (PFTs) to quantify the lung function and extent of disease. A lung biopsy may be necessary in some cases, and sometimes a bronchoscopy may need to be done to rule out an infection.

While rare, lupus can affect the blood vessels in the lungs by causing what is called pulmonary hypertension, or literally high blood pressure within the blood vessels in the lungs. Symptoms of this disease entity may include shortness of breath, particularly with exertion, chest pain, fatigue, weakness, and even palpitations. Your doctor will need to listen to you heart in addition to your lungs to listen for some changes in heart sounds that may occur. Sometimes an ultrasound of the heart can help with this diagnosis before other invasive testing.

A rare disease entity that’s unique to lupus includes acute lupus pneumonitis, caused by inflammation in the lungs, and may cause shortness of breath, rapid breathing, cough, and even coughing up blood. Again imaging would be needed and your doctor would have to make sure there are no infections present causing this problem.

These are only some of the many lung disorders that can occur in lupus and some others will be discussed below. If you have symptoms of difficulty breathing, shortness of breath, pain while breathing , coughing, or increasing shortness of breath while exercising or chest pain then discuss them with your doctor in detail so they can decide what further testing might be needed.

2. Can you explain the connection between Lupus and shrinking lung syndrome? Decatur, IL

Shrinking or "Vanishing" lung syndrome has been seen in patients (both adults and kids) with lupus and has been characterized by symptoms of shortness of breath, chest pain while breathing in, and literally a progressive decline in the size of the lungs on your breathing studies (PFTs). In addition, there may be disease of the lining around the lung that may be seen on a CAT scan and diaphragm muscles look like they are moving upwards because they are weak. While it's not clear why this happens some research suggests this is from muscle dysfunction and weakness of the diaphragms (the major breathing muscles). This may happen from inflammation in the diaphragm muscles itself from lupus. Sometimes steroids or other immunosuppressive therapy can therefore help treat this problem.

3. Has Bronchiolitis Obliterans with Organizing Pneumonia (BOOP) ever been associated with Lupus? How long does it usually take prednisone treatment to ease symptoms? Highland Beach, FL

BOOP, which is also called Cryptogenic Organizing Pneumonia (COP) is not a common pulmonary manifestation of lupus, but can in fact be associated with lupus. It is a disease entity caused by inflammation of the airways, or bronchioles and surrounding tissue in the lungs. Treatment usually includes prednisone and it may takes months to fully improve and the treatment course may be anywhere for 4 months to a year. Some patients need even more aggressive immunosuppressive therapy if prednisone doesn’t work.

4. During a flare several years ago, a chest x-ray showed nodules in my lungs. After the flare and months later another x-ray showed a normal lung. Can nodules in the lungs come and go with lupus flares? Greensboro, NC

Nodules can be a sign of inflammation in the lungs and are more commonly seen in Rheumatoid Arthritis, but can be seen in lupus. Nodules or other subtle abnormalities on an x-ray can come and go with lupus flares. I would think of this as a manifestation of the inflammatory disease in the lungs.

5. How common are pulmonary lesions with lupus? What is their overall significance? Boynton Beach, FL

The pulmonary manifestations of lupus are so broad that it’s tough to give a number on how common this is. Some manifestations are more common than others. The most common are pleural manifestations and chest discomfort which can occur if up to 50% of patients with SLE. This is much more common than something like lupus pneumonitis which affects about 5% of patients with lupus. That being said, most patients will have some pulmonary manifestation of lupus at some time during their disease course, even if very mild. Some pulmonary problems are much more severe than others and require much more aggressive treatments. If you have a breathing symptom, it’s important to bring it up with your doctor.

6. Is there an increased risk of developing COPD when you have lupus? St. Louis, MO

COPD or Chronic Obstructive Pulmonary Disease is generally a disease of smokers that affects the airways of the lungs. Some smokers also get emphysema which is actual destruction of the lung tissue from smoking that causes something like holes in the lungs. In a small number of lupus patients, it has been noted that they develop some narrowing of the airways and airflow limitation, and this is found to be strongly associated with smoking. However some patients with lupus may develop this without smoking at all. The treatment includes inhalers that we use to treat COPD. Certainly if one has lupus and smokes, I always recommend quitting smoking.

7. Does pre-existing asthma increase the risk of lupus in the lungs? Holt, MI

As far as I know pre-existing asthma doesn’t increase the risk of lupus affecting the lungs. Asthma is an airway disease which also causes airflow limitation. As with the symptoms are mentioned above, lupus can also cause airflow limitation and wheezing and the treatments include inhalers.

8. If you already have asthma/COPD, how do you know if lupus is making it worse, or causing new problems? Fredericksburg, VA

This is a difficult one as often times it can be difficult to differentiate what is from lupus and what is from asthma or COPD. Sometimes breathing studies of PFTs may be useful, and a CAT scan to look at the lungs may help your doctor differentiate from some other problems.

Asthma and COPD also tend to have triggers, like seasonal allergies, weather changes, scents, infections, and those triggers would more often make asthma or COPD worse rather than lupus itself.

9. Since having been diagnosed with systemic lupus my lungs have become very jittery as my Pulmonologist would call it. I come down with status asthmatics very quickly and for unknown reasons. We usually find out later that I am nursing some infectious process either viral or bacterial. The issue is that I don't even feel sick prior to these episodes. This has only happened since I was diagnosed with SLE. Can you give me some insight? Newbury Park, CA

This may be because you take immunosuppressive therapy which may mask the symptoms of an infection. For example, someone who takes chronic prednisone may not get a fever when they have an infection. If you have underlying asthma and get an infection that affects the lungs, the airways may be very sensitive and reactive which flare up quite easily. Since you have lupus, your body may also be more prone to inflammation which also affects the lungs, and therefore your lungs flare up much more aggressively and quickly then they may have in the past. Some people have asthma and don’t even know it until they have a flare up. I think what would be most helpful in your case is to have you on chronic therapy for asthma, usually inhalers, and try to avoid infection by good hand washing and avoid being around anyone sick.

10. Unfortunately, I have been a smoker now for 22 years. I was diagnosed with lupus in July 2011 and after numerous attempts to quit smoking, all have been failures. I still plan to quit ASAP, but in the meantime, will this cause lupus to affect my lungs more rapidly? Ashdown, AZ

As I mentioned before lupus can cause airflow limitation and narrowing of the airways and this is much more common in smokers with lupus. I don’t know if lupus may affect the lungs more rapidly due to smoking but I do recommend quitting smoking as you may have some damage already to the lungs from smoking limiting your lung function. Quitting smoking which can be one of the most difficult challenges that someone faces when they've been smoking for years and have had a new stressor introduced to their life: being diagnosed with lupus. There are several aides we use to help people quit smoking. Sometimes nicotine supplementation helps with the gum or the patch if you are strongly addicted to the nicotine. We also offer medications in pill form that help cut the craving for smoking. This includes a medication called Zyban and a medication called Chantix. You'd have to see your doctor to see if you may be a good candidate for these sorts of therapies as they're not for everyone. Some people find alternative therapies like biofeedback or hypnosis helpful. I strongly recommend trying a support group with others who are trying to quit so that you know you're not alone. In California, there is a smoker’s hotline that one can call for free where they can help assist you with the different services available and counseling. See if there may be something like this where you live.

11. What is a ground-glass opacity appearance? A previous CT of my lungs indicated this but seems to be clear now. Grayslake, IL

A "ground glass" appearance is a description of what was seen on a CAT scan of your lungs. This is haziness that the radiologist describes which usually represents inflammation in the lungs. It's called ground glass because it literally looks like a piece of glass that’s surface has been ground to give it a rough finish, so if you tried to look through it would be hazy, rather than a piece of clear glass like your window or windshield one would look through. If you had this finding on a CAT scan it most likely represented inflammation and was treated by treating your underling lupus. Sometimes excess fluid in the lungs can give this appearance as well and this may have resolved

12. Are blood clots or spontaneous bleeding (alveolar hemorrhage) of the lungs possible or common in someone with lupus? Dallas, TX

Yes, both blood clots and/or spontaneous bleeding can occur in the lungs in those with lupus, but both are rare.

Some people with lupus may have what is called Antiphospholipid antibodies. You can be checked for this with a blood test. People with these antibodies may have a propensity to develop blood clots. Blood clots may occur anywhere in the body but most commonly occur in the legs or lungs. Some people need to be placed on blood thinners to avoid developing clots.

Pulmonary hemorrhage or bleeding into the lungs is a very rare and dangerous pulmonary complication of lupus. Patients with this problem are acutely ill and usually present with symptoms of cough, shortness of breath and coughing up blood. This requires hospitalization and tests to make sure there is no infection causing these symptoms and to receive aggressive treatment. Again, this is very rare.

13. It has been explained to me that because of a heart valve problem, I have pulmonary hypertension, not sever yet. Can you provide some information on pulmonary hypertension and are there steps that I can take to keep the situation under control and if so what are they? Merced, CA

Pulmonary hypertension is a problem where the pressure in the pulmonary artery, which is attaching the right side of the heart to the lungs, is elevated. Pulmonary hypertension is associated with lupus but it’s not clear if your heart valve problem came first or if the pulmonary hypertension is causing the valve problem. Not everyone with pulmonary hypertension needs treatment, but if it is progressing and getting worse it may need treatment. I recommend eating a low salt diet, and staying on your lupus treatment. In addition, if you are overweight then weight loss may help, and if there is any suspicion that you have sleep apnea that should be checked for and treated. However, if you have pulmonary hypertension that requires treatment, I recommend being seen at a center that specifically treats this problem. Going to the PAH (pulmonary arterial hypertension) website may help with more information.

14. Have been diagnosed with interstitial lung disease. Blood oxygen in the 85 to 92 range. Have had SLE for 10 years. Minor symptoms until now. Was taking plaquenil and now my doctor has added CellCept. Could the CellCept reverse or improve the lung condition? Jacksonville, FL

I agree that if you have interstitial lung disease as a manifestation of your lupus you need some more aggressive treatment in addition to plaquenil. The CellCept and other drugs like it are used to help suppress the immune system from causing more damage to the lungs. Depending on what kind of interstitial lung disease you have and how much scarring is present in the lungs, the CellCept may help reverse some of it, but mostly it will hopefully help this disease entity from progressing in the lungs. Your lung function needs to be followed carefully and on a regular basis if you have interstitial lung disease to make sure it isn’t progressing.

15. Are there supplements that can be taken to support the lungs? What other preventive measures are possible? Murrieta, CA

Unfortunately we don’t really have any good supplements to support the lungs that I can officially recommend. The supplement N-acetylcysteine or NAC, which is an antioxidant, is being studied in some people with interstitial lung disease, but the verdict isn’t in yet whether it alone may be helpful in the lungs. Certainly, there are plenty of other doctors who may disagree with me and give you a long list of supplements that they recommend for lung health. If you're going to take any of these, please check with your rheumatologist first to make sure nothing interacts with your lupus medication. While they may not need a prescription, supplements are drugs too and have side effects!

Personally I think that physical exercise is the best thing for the lungs, and avoiding infection of course. Whatever you're able to do physically with your lupus, even just walking, is good exercise for the lungs!