Rheumatoid arthritis is a chronic, inflammatory autoimmune disease most commonly associated with severe, often disabling destruction of the joints. But the damage spawned by RA is systemic and widespread, attacking many of the body’s organs, including the heart, eyes, skin and blood vessels and shortening the lifespan of individuals with RA by approximately 10 years. RA can also wreak substantial havoc on the lungs. According to the Arthritis Center at Johns Hopkins University, respiratory diseases are the second leading cause of death in people with RA.

Over the past two decades, the development of medications like disease-modifying anti-rheumatic drugs, corticosteroids and biologics — medications specifically engineered to inhibit many of the inflammatory compounds of the immune system triggered by RA — have substantially lowered disease activity and improved the overall prognosis in many people afflicted by the disease.

Unfortunately, these benefits have not extended to RA-associated lung disease.

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RA affects the lungs in a number of ways. The most common, and most serious, of these is interstitial lung disease, which leads to scarring, or pulmonary fibrosis, of lung tissue, including the network of cells involved in processing oxygen.

These changes to the lungs can be ongoing for years before they show up on X-rays or cause symptoms of decreased lung capacity hallmarked by restricted breathing or shortness of breath. However, once ILD becomes clinically apparent, it is associated with significant mortality.

Another pulmonary manifestation of RA is chronic obstructive pulmonary disease, which includes chronic bronchitis or emphysema, or both. According to Dr. Dimitrios Pappas, a rheumatologist at New York Presbyterian, COPD occurs more frequently in individuals with RA than in the general population, even after adjusting for smoking,

“It is not exactly clear why the lungs are affected in rheumatoid arthritis,” says Dr. Teng Moua, who is the director of the Interstitial Lung Disease Clinic at the Mayo Clinic in Rochester, Minnesota. “Epidemiologic studies suggest that between 6 to 10 percent of patients with RA develop some form of ILD,” he adds.

People with RA and lung disease have a poorer prognosis than individuals who do not have RA but have lung disease. “It is generally recognized that having RA with ILD bodes a poorer course than having RA alone,” Moua says.

Diagnosis of both ILD and COPD is often delayed because the early signs and symptoms are not specific and may not be apparent because many people with RA are generally inactive due to joint pain and general fatigue. So they are less likely to experience shortness of breath with activity.

Interestingly, although women are about three times more likely to have RA than men, lung disease is somewhat more prevalent in men with RA. “We don’t really understand why men have slightly more risk of developing ILD in RA,” Moua says. “Some have suggested that the greater prevalence of smoking among men explains the gender difference, especially since smoking is also associated with greater risk of developing ILD in RA.”

Smoking is a known risk factor for the development of ILD in RA patients, particularly in people who have smoked for many years or smoke a greater number of cigarettes. ” Quitting smoking early may reduce the risk and severity of ILD associated with RA, though its effect on disease progression or response to therapy after ILD has already developed is not well understood,” Moua says.

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Along with male gender, older age and more severe RA disease have been reported as risk factors for developing ILD, Moua adds.

Another potential cause of ILD in people with RA is the use of certain medications used to treat the disease. “Some studies have suggested the use of methotrexate and other RA drugs as possibly leading to more lung injury, though this has been less agreed upon in recent years,” Moua explains.

“Other genetic or inflammatory markers seen in blood work have been shown to be associated with ILD, though this is inconclusive,” he adds.

Is it possible to decrease the risk of lung disease in people with RA? According to Moua, stopping smoking and closely monitoring the underlying RA disease are probably the only currently modifiable risk factors. But these interventions may only help to a point.

“Lung disease associated with RA may act independently of the systemic process, meaning that patients may attain good control of joint or other constitutional [RA] symptoms, but still have flares or progression of lung disease,” Moua says. “Close monitoring and directed treatment is often needed to maintain lung function over time.”

“The optimal treatment for ILD associated with RA has not been well-studied and is based on reviews of historic treatment regimens, ” says Moua, who notes that ILD associated with RA is often treated independently of the underlying RA process.

“In general, treatment for ILD often involves prednisone or a prednisone-sparing agent such as mycophenolate or azathioprine [administered] over several weeks to months, with immediate improvement often seen within that time frame,” he says. “This is defined as resolution or decrease in respiratory symptoms, and stability or [improved] lung function as tested formally in the pulmonary clinic.”

In some people, a lung transplant may be necessary. “This is an option for people with more advanced and unresponsive disease who meet selected criteria for transplantation,” he says.

If you have RA and lung disease, what do you need to know? According to Moua, the diagnosis and long-term management of ILD in RA often requires collaboration between a pulmonologist and rheumatologist. “Because established therapy for RA-ILD is not well-defined, close communication between the two is needed to best monitor disease progression and decide on the best course of therapy,” he says. This is often done on an individualized basis, because disease course and response to therapy is often unique to each patient.

This collaboration is essential to minimize side effects and potential harm and maximize the benefits of treatment for both conditions, he adds.

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The Arthritis Foundation also recommends that you get pneumonia and flu vaccinations because people with RA taking immune-suppressing medications are at higher risk of these diseases.

It may be possible that medical intervention at an early stage may be beneficial, according to Pappas, who is an assistant adjunct professor of medicine at Columbia University School of Medicine. “Early diagnosis and treatment with antifibrotic agents may alter the prognosis of pulmonary fibrosis,” he says. Prompt intervention in patients with RA and lung disease can potentially improve overall quality of life and lung performance, he adds.

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People With Rheumatoid Arthritis Have a Risk of Interstitial Lung Disease originally appeared on usnews.com