Systemic lupus erythematosus (SLE) can cause severe pain and permanent damage to multiple organ systems, including joint tissue, skin, heart, lungs, blood vessels, liver, kidneys, and the nervous system. Prevalence has been estimated to range from 52 to 150 per 100,000 individuals in the United States, with 80% to 90% of cases occurring in women between 20 and 40 years, and a much higher prevalence in people of African American, Afro-Caribbean, or Asian ethnicity.1,2
Median survival rates have improved over recent decades; 95% of patients now reach 5-year survival, 85% reach 10-year survival, and 75% reach 20-year survival.2,3 Recent developments in diagnosis and treatment have led to an increased focus on improving quality of life (QoL) in SLE. “Some of the factors that affect QoL include fatigue, depression, poor sleep, pain, poor coping, and lack of social support,” explained Michelle Petri, MD, director of the Johns Hopkins Lupus Center at Johns Hopkins Medical Center in Baltimore, Maryland, in an interview with Rheumatology Advisor.
Quality of Life Issues
In recent studies that used the Medical Outcomes Study Short Form 36 (SF-36) as a specific measure of health-related quality of life (HRQoL), patients with SLE consistently scored significantly lower than the general population.4,5 Furthermore, scores of patients with SLE indicated a much worse HRQoL compared with other chronic diseases such as AIDs, rheumatoid arthritis, diabetes, and congestive heart failure.4
“Although there is a poor association between activity and damage with QoL, it is evident that QoL measurement captures unique information not included in disease activity or damage evaluations. QoL generally captures distal effects of symptoms (eg, pain),” Meenakshi Jolly, MD, MS, director of the Rush Lupus Clinic at the Rush University Medical Center in Chicago, Illinois, told Rheumatology Advisor. “In a [patient with lupus], symptoms of lupus from active disease, or the medications taken for treatment of lupus, can result in impairments in physical, emotional, and social functioning, as well as pain, fatigue, and poor sleep, among others. Patients with lupus may have a poor body image resulting from active lupus symptoms or the treatment,” she said.
The multitude of symptoms in SLE has a significant effect on HRQoL, with different composite pictures in individual patients. “In my experience, the most important factors contributing to QoL differ from individual to individual. Common problems my patients identify as interfering with life include fatigue, pain, and skin and hair changes,” reported Stacy Ardoin, MD, a clinical rheumatologist at the Ohio State University Wexner Medical Center in Columbus, Ohio.
Dr Petri pointed out that most patients “have 2 major complaints — chronic fatigue and pain — that don’t correlate well with lupus activity,” and this is a phenomenon that is commonly reported in the literature.4-8 While factors including older age, the presence of fatigue, or comorbid psychological disorders such as depression or anxiety have been found to closely correlate with objective measures of HRQoL, clinical measures of disease activity and organ damage are widely considered poor indicators of patient perceptions of QoL.4,5,7
Emotional distress is increased in patients with SLE as a consequence of many factors, including embarrassment about skin and hair changes, anxiety caused by disease flares and concern about how their disease may progress in the future. “Depression is very common in SLE— around 30% [of patients]— and it is affected by some subsets of lupus manifestations such as spinal cord involvement and cutaneous lupus, which can be disfiguring, but also by prednisone, which increases depression,” Dr Petri reported. “We have shown that the cumulative effect on QoL in [patients with lupus] may be worse than patients with congestive heart failure or a major depressive disorder,”8 Dr Jolly said.
Nonspecific pain and fatigue are the most common symptoms of fibromyalgia, which often coexist in patients with SLE, making it difficult to determine the cause.7,9 “Up to one-third of [patients with lupus] may have concomitant fibromyalgia,” Dr Jolly stated, explaining that they “frequently have pain, fatigue, poor sleep, stress, anxiety, or depression. These kinds of symptoms may also be seen with lupus.” Dr Petri agreed, noting that, “30% of my SLE patients also have fibromyalgia. Often the fibromyalgia is more of a day-to-day problem than SLE. There is frustration when I say the ‘lupus is under control’ but the patient is not feeling well due to chronic fatigue and pain.”
“As with many rheumatic diseases like rheumatoid arthritis, psoriatic arthritis, and others, there are some people who definitely also struggle with fibromyalgia,” Dr Ardoin told Rheumatology Advisor. “Chronic pain like fibromyalgia pain can make it harder to do things and can affect mood. It can also become a barrier to healthy behaviors like exercise, which in general can improve mood and quality of life.”
Helping patients separate symptoms of fibromyalgia from SLE is often a treatment challenge, as the pharmacologic therapies for SLE do not have an effect on fibromyalgia symptoms. “I spend just as much time managing fibromyalgia in some SLE patients as I do managing their lupus,” Dr Petri said. “In the clinic, I ask patients what is bothering them and I listen. There is no substitute for clear and open communication with the patient — but sometimes they don’t hear me when I explain that fibromyalgia is a chronic pain sensitization syndrome that is not going to be helped by SLE medications.”
“I do use both non-approved and FDA [US Food and Drug Administration]-approved medications for fibromyalgia pain — but never narcotics, as they worsen chronic pain,” Dr Petri explained, adding that, “Fibromyalgia management needs to emphasize education and motivate patients to invest time in stretching and aerobic exercise.” The literature supports the benefits of exercise for fatigue associated with SLE; studies have demonstrated that the majority of patients with fatigue improve with exercise interventions, regardless of type of exercise of level of compliance.10,11
Managing Patient Perceptions
A major contributing factor to emotional distress and the perception of reduced QoL in patients with SLE is the diagnosis itself. “When lupus is first diagnosed, it can be pretty overwhelming and the disease itself is often active, causing a lot of symptoms,” stated Dr Ardoin. “People react to this stress in different ways, but certainly a new reality of having a serious chronic disease and having to take medicines and go to many appointments has a negative impact on quality of life.”
Dr Jolly noted that “patients with flares and active lupus may experience significant concerns about lupus symptoms, medications, pain, fatigue, or emotional health. Their expectations may be targeted towards any improvement, albeit small, in their day-to-day concerns, and they are thus anchored in their current disease activity state. These changes may be achievable over shorter periods of time with better control of the flare through institution of appropriate treatment. As the patient’s disease is better controlled or when they are in remission, their focus moves to getting their QoL back to their baseline before lupus, and this may not always be possible,” she said.
Perception of QoL in SLE has been noted to improve over time, regardless of disease status, Dr Ardoin noted, because “in most people, lupus can be managed to the point where it is a nuisance rather than the main attraction. In that phase, the quality of life usually seems to be better, but there can still be problems relating to medication side effects, intermittent disease flares, pain, fatigue, and impact on mood,” she said.
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