People with Certain Medical Conditions

Understanding The Connection Between Lupus And The Lungs

Cheryl Russell of Highland Beach, FL, doesn't indulge in what she calls "symptom chasing." After more than three decades with lupus, she refuses to head to the doctor every time she has joint pain or some other problem.

"I learned early on that if I do that, I just end up going to specialist after specialist and having test after test, chasing symptoms that will disappear on their own," says Russell, who raised two now-grown children with her husband, Jake. "I let things have time to work themselves out before jumping on them." 

Trouble began in June 2011 when she noticed she could no longer take a deep breath. She couldn't yawn, and sneezing felt weird. She didn't say anything to her husband, her children, or her doctor. By July, her breathing was worse, and her feet started to swell. Still Russell stayed silent, unaware of the possible connection between lupus and lung disorders and intent on not spoiling a road trip to New England.

The couple had almost made it home from their three-week vacation when things came to a head. In the middle of the night in South Carolina, Russell woke up in their hotel room with intense chest pain and extreme difficulty breathing. She was intent on seeing her own doctors, so the couple hightailed it—so fast they got a speeding ticket—to the emergency room at Boca Raton Regional Hospital. The cause of Russell's breathing difficulties and her swollen feet was a potentially deadly blood clot in her lungs. She also had a rare lung condition called bronchiolitis obliterans with organizing pneumonia, or BOOP.

After eight days in the hospital, doctors sent Russell home with prescriptions for prednisone, a blood thinner, and an amped-up dose of the immunosuppressive drug CellCept®. Although Russell is now doing better, she still can't walk and talk at the same time.

The whole experience has been eye-opening. "Even for someone like myself who's been involved with lupus for so many years, I had never heard of anyone having any lupus-related lung problems," says Russell, who hopes a regimen of Benlysta® infusion therapy will let her get back to her favorite pastimes of cooking and gardening.

It surprises many people to learn that lung issues are common among people with lupus. According to the Johns Hopkins Lupus Center, about 50% of people with lupus will experience lung issues during the course of their disease.

In fact, lung complications rank just behind problems with joints, skin, and blood cell counts, says Ben deBoisblanc, M.D., a professor of medicine and physiology at the Louisiana State University Health Sciences Center in New Orleans.

Research has shown that at some point, most people living with lupus will show signs of lung involvement. "The more severe the lupus you have, the more likely you are to have lung problems," he says. 

Lung involvement in lupus

Although the underlying connective tissue disease is the root cause of lupus-related lung problems, the exact mechanism differs for each of the most common conditions.

The most frequent lung problem that affects people with lupus is pleuritis, also known as pleurisy. In this condition, the pleura—a membrane that covers the exterior of the lungs and the interior of the chest cavity—becomes inflamed. Although the pleura normally produces a small amount of fluid to lubricate the space between the chest wall and the lungs, that fluid can build up excessively. Pleural effusion, which occurs when the fluid between the lungs and the chest wall leaks out, can cause a lot of pain.

With lupus pneumonitis, the inflammation is within the lung tissue itself. The cause is usually an infection by bacteria, viruses, or fungi. It can also be caused by an autoimmune injury to the lung, deBoisblanc adds.

Blood clots in the lungs, known as pulmonary emboli, present another possible complication. "Lupus can cause blood clots to form and break loose and go to the lungs," deBoisblanc explains.

Some people with lupus have a condition called antiphospholipid antibody syndrome that puts them at even higher risk of pulmonary emboli. With this disorder, the body produces antibodies against normal blood proteins, which can cause clots to form in the arteries.

Less common—except among people who have both lupus and other types of connective tissue disorders such as scleroderma—is pulmonary hypertension, or high blood pressure in the blood vessels within the lungs.

The disorder is still a bit mysterious, says Steven M. Kawut, M.D., director of the pulmonary vascular disease program at the Perelman School of Medicine at the University of Pennsylvania. 

"We don't know the pathophysiology," he says. What doctors do know is that the high blood pressure in the lungs' blood vessels strains the right side of the heart and can cause shortness of breath, swelling of the lower extremities, and, eventually, heart failure.

Other lung complications include chronic diffuse interstitial lung disease, which can scar the lung and prevent oxygen from moving easily from the lungs into the blood; pulmonary hemorrhage, or bleeding into the lungs; and "shrinking lung" syndrome, which causes a sense of breathlessness and the feeling that the lungs are not able to expand.

Know the signs

The good news, according to lung specialists, is that diagnosing these potentially life-threatening conditions is usually straightforward, and treatment is successful for most conditions—especially if caught early. A pulmonologist can help with testing and diagnosis, but often a rheumatologist directs treatment.

When you have lupus, you should watch carefully for signs of pulmonary trouble. Keep an eye out for two common symptoms: shortness of breath and pain with breathing, deBoisblanc says. "Pulmonary issues often affect people with lupus who are younger, and who shouldn't be having significant breathing problems at all," he says.

Of course, each lung condition has its own symptoms and diagnostic methods. For example, a sharp pain in the chest that worsens during deep breathing, coughing, or even laughing suggests pleuritis, which a chest X-ray can confirm.

X-rays can also be useful in diagnosing pneumonitis, which may be suspected if you're coughing a lot, experiencing chest pain and shortness of breath, and running a fever. Your doctor may also order tests of your blood and sputum—the mucus from the lungs. A bronchoscopy looks inside your lungs to see if an infection is the pneumonitis' cause.  

The unexplained shortness of breath, fainting, and chest pain of pulmonary hypertension can be picked up via echocardiography and then right-heart catheterization. Your doctor will first rule out other possible causes, such as sleep disorders and other lung diseases, Kawut says. Chest pain and shortness of breath can also mean pulmonary emboli, as Russell discovered.   

For Paula Rostron of Killingly, CT, lung problems actually led to her lupus diagnosis. In 2004, Rostron noticed she was having palpitations and was out of breath, even when doing something as non strenuous as blow-drying her hair. Her doctor did an EKG and immediately sent Rostron to the hospital after seeing the results. There, she was diagnosed with pulmonary emboli. "Even a tiny blood clot can kill a person, and I had huge blood clots in my lungs," she remembers.

Rostron spent a week in intensive care, then went home with a prescription for blood thinners and was soon diagnosed with antiphospholipid antibody syndrome. She was diagnosed with lupus in 2010, after experiencing migraines, joint pain, and pleuritic symptoms.

Treating lupus and the lungs

Treatment should focus on both the underlying lupus and the specific lung problem, deBoisblanc says. For pleuritis, that means immunosuppressive drugs plus analgesics to soothe the pain. For pneumonitis, treatment typically begins with antibiotics to rule out infection, followed by corticosteroids and immunosuppressants such as Imuran®. Pulmonary emboli necessitate blood thinners, often indefinitely.

Treatment for pulmonary hypertension shows the progress being made in treating lupus-related lung problems.

"Twenty years ago, there were no therapies for pulmonary hypertension," says deBoisblanc, and life expectancy was very limited. "Now we have excellent therapies that allow patients to manage their pulmonary hypertension not too differently than you'd manage any chronic disease."

Renee Mestayer, one of Dr. DeBoisblanc's patients, witnessed the evolution of treatments firsthand. Diagnosed with lupus in 1983, Mestayer, of New Iberia, LA, developed pulmonary hypertension in 1999. Eventually, she could barely walk to the mailbox in front of her house and had to use an oxygen tank.

Mestayer has tried a number of new medications over the past decade in addition to her lupus medications. She now takes the pulmonary hypertension drug Tracleer®, as well as Revatio®, which relaxes the walls of arteries and lowers blood pressure, and Tyvaso®, which also helps keep arteries open.

"I'm very fortunate because of all the new medicines that have come out in the last 10 years," says Mestayer. Although she still tires easily, she can now walk long distances on her treadmill. She credits her medications and her faith for getting her through the experience.

"At the time I was diagnosed, my two boys were in sixth and seventh grade," says Mestayer. "I asked God to let me see them graduate high school—and now I'm a grandma!"

How Lupus Affects The Lungs And Pulmonary System

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.

Pleuritis

The most common way that lupus can affect your lungs is through inflammation of the pleura, the lining that covers the outside of the lungs. The symptom of pleuritis that you may experience is severe, often sharp, stabbing pain in a specific area or areas of your chest. The pain, which is called pleurisy, is made worse when you take a deep breath, cough, sneeze, or laugh. You may also experience shortness of breath. Sometimes an abnormal amount of fluid will build up in the space between your lungs and your chest wall; when it leaks out it is called a pleural effusion. Pain from pleurisy, with or without effusions, is found in 40 to 60 percent of people with lupus.

Pneumonitis

The term for inflammation within the lung tissue is pneumonitis. The symptoms of pneumonitis that you may experience are fever, chest pain, shortness of breath, and cough. An infection caused by bacteria, virus, or fungi is the most common cause of pneumonitis.

Chronic diffuse interstitial lung disease

When inflammation in the lungs is chronic, it can cause scarring. This scar tissue can prevent oxygen from moving easily from your lungs into your blood and may cause diffuse (widespread) interstitial lung disease. The symptoms that you may experience include a chronic dry cough, chest pain, and difficulty breathing during physical activity.

Pulmonary emboli

Blood clots that block the arteries leading to the lungs are called pulmonary emboli. These blood clots will cause chest pain and shortness of breath, but can also lead to a decrease in oxygen flow in your lungs. You are at increased risk for pulmonary emboli if you have antiphospholipid antibodies, vascular damage, and/or an inactive lifestyle.

Lupus Expert Debunks 7 Common Myths About The Autoimmune Disease: 'Not A Death Sentence'

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Fatigue, pain, swelling, rashes and hair loss are just some of the symptoms that affect people with lupus, a chronic autoimmune disease in which the immune system attacks healthy tissue.

Some 1.5 million Americans are living with lupus, with about 16,000 new cases each year, according to the Lupus Foundation of America, based in Washington, D.C.

There are many myths surrounding lupus that can make it difficult for people to understand and manage the disease, according to Dr. Brooke Goldner, a board-certified medical doctor and an autoimmune professor at Cornell University.

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"It's essential to educate yourself and others about lupus to dispel these myths and increase understanding of the disease," Golder, who was diagnosed with lupus at the age of 16, told Fox News Digital.

For Lupus Awareness Month, Goldner shared some of the biggest misconceptions — and set the record straight on a number of issues.

Dr. Brooke Goldner, a board-certified medical doctor and an autoimmune professor at Cornell University, pictured at right, is committed to debunking lupus myths and misconceptions. (iStock/Dr. Brooke Goldner)

Myth No. 1: There is only one type of lupus

The most common type of lupus is systemic lupus erythematosus (SLE), but it's not the only form of the disease. 

"SLE can have a wide range of symptoms that may come and go, making it challenging to diagnose," Goldner said. 

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Some of the common symptoms of SLE include fatigue, joint pain and stiffness, fever, hair loss, skin rashes and sensitivity to sunlight.

Cutaneous lupus erythematosus (CLE), a less common form, affects only the skin. 

The two least common types are neonatal lupus and drug-induced lupus, according to the Lupus Foundation of America.

Myth No. 2: Lupus is contagious

Lupus cannot be transmitted from person to person, Goldner said. 

"It occurs when your immune system attacks your own tissues and organs, causing inflammation and damage," she said. 

"Lupus can affect various parts of the body, including the skin, joints, kidneys, brain and other organs," one doctor said. (BSIP/Universal Images Group via Getty Images)

"Lupus can affect various parts of the body, including the skin, joints, kidneys, brain and other organs."

Myth No. 3: Lupus only affects women

"While lupus does affect more women than men, it can affect anyone, including children and men," Goldner said. 

Anyone can develop lupus. Yet 90% of cases affect women between the ages of 15 and 44, according to the Centers for Disease Control and Prevention (CDC).

Myth No. 4: Lupus is a cancer

Medicines like chemotherapy are often used in severe lupus cases, but it is not a form of cancer. 

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"It is an autoimmune disease, whereby the immune system begins attacking the body's own tissues rather than just foreign invaders like viruses and bacteria," Goldner told Fox News Digital.

"Chemotherapy is known as an immune system suppressant, which can be lifesaving when lupus is causing organ failure and aggressive immunosuppression is required."

Myth No. 5: Lupus is caused by stress

While stress can trigger lupus symptoms, Goldner noted it is not the cause of the disease.

"The exact cause of lupus is unknown, but it is believed to be a combination of genetic, environmental and hormonal factors," she said.

Myth No. 6: Lupus is purely caused by genetics

Genetics will determine whether you have the possibility of developing lupus, but it is not a condition you are born with, according to Goldner. 

Fatigue is a primary symptom of systemic lupus erythematosus (SLE). (iStock)

"Just like someone with the genetics to become type 2 diabetic will not develop the disease unless they have a diet and lifestyle that triggers it, the same is true for lupus," she said.

Lupus is often triggered during times of physical and emotional stress combined with a nutrient-poor inflammatory diet, the expert added.

Myth No. 7: Lupus is a death sentence

While lupus can be a serious disease, it is "not a death sentence," according to Goldner. 

"While there is no medical cure for lupus, there are treatments available that can help manage the symptoms and prevent damage to vital organs," she said.

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"Treatment may include medications such as anti-inflammatory drugs, immunosuppressants and corticosteroids." 

In addition to taking medications, many people with lupus can manage symptoms through healthy lifestyle interventions, according to Goldner.

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"Lifestyle changes such as regular exercise, healthy eating and stress management can help improve the quality of life for people with lupus," the expert said.

"Lifestyle changes such as regular exercise, healthy eating and stress management can help improve the quality of life for people with lupus," the expert said. (iStock)

As a survivor of lupus and a physician, Goldner said she has dedicated her life to bringing more awareness to the disease and helping people gain the power to manage and eliminate symptoms through nutrition and lifestyle.

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"This is not to suggest that people should not use medical treatments that can be lifesaving," she said, "but rather that they embrace taking control of all the variables they can manage, like how they eat, sleep and manage stress with self-care, so they can minimize illness and maximize recovery and remission."

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