Systemic lupus erythematosus (SLE) is a leading cause of death among young women, according to an August 2018 study in Arthritis & Rheumatology.1 To help determine where SLE ranks among causes of death, Eric Y. Yen, MD, and Ram Raj Singh, MD, conducted a population-based study using nationwide mortality counts for all female residents of the U.S. from 2000 to 2015. They concluded that SLE is an important public health issue among young women and that ongoing research and education are needed to address it.
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Explore This IssueOctober 2018
“During my medical training in pediatrics, I noticed that lupus is a common disease that I see on the wards,” says Dr. Yen, assistant professor, Department of Medicine, University of California, Los Angeles (UCLA). “So when I joined the pediatric rheumatology fellowship at UCLA, I set out to determine SLE death rates.”
“To our dismay, we found the death rates for SLE were disproportionately high relative to death rates in the general population. … This finding led us to posit that SLE might be among the leading causes of death,” explains Dr. Singh, professor of medicine and pathology, and director of the Autoimmunity and Tolerance Laboratory, UCLA.
Previously, Drs. Yen and Singh, along with several others, conducted a multiple-regression analysis of SLE mortality risk stratified by race and ethnicity, which was published in the Annals of Internal Medicine in 2017. The analysis showed that SLE mortality risk was higher for women than men of all races and ethnicities, with the greatest differences among black and Hispanic patients.
Dr. Yen also noticed that SLE was not included in the 113 diseases on the Centers for Disease Control and Prevention’s (CDC’s) ranked list of leading causes of death. “Given my suspicion that SLE was more common than some of the other diseases on the CDC list, I decided to take on this project,” he says of the current research.
Study Methods & Results
Drs. Yen and Singh conducted a population-based study using nationwide mortality counts for U.S. girls and women. They obtained data on SLE deaths from the CDC Wide-Ranging Online Data for Epidemiologic Research (WONDER) database. They attributed deaths to SLE if codes from the International Classification of Diseases, 10th revision (ICD-10), for SLE were listed as an underlying or contributing cause of death on the death certificate: M32.1, SLE with organ or system involvement; M32.8, other forms of SLE; and M32.9, SLE, unspecified.
The researchers found that SLE is among the top 20 leading causes of death in females aged 5 to 64 years. The deaths of 28,411 girls and women were recorded with SLE as the underlying or a contributing cause of death between 2000 and 2015. The largest number of SLE deaths was among women aged 65 years and older (10,238). In women aged 15–24 years, SLE was the top cause of death among chronic inflammatory diseases, more so than diabetes mellitus, HIV, chronic lower respiratory disease, nephritis, pneumonitis and liver disease.
Although SLE was shown to be among the leading causes of death in young girls and women, it may actually rank higher. Previous research showed SLE may not be recorded on the death certificate for as many as 40% of patients with SLE in the U.S. This under-reporting may be a result of patients with SLE dying prematurely of complications, including cardiovascular events, infections, renal failure and respiratory diseases.
Additionally, at the time of death, many SLE patients may be under the care of physicians who may have a limited awareness of SLE as the underlying cause of death. For example, one Swedish study showed 86% of 2,314 SLE deaths in Sweden occurred in hospital units other than rheumatology.
Implications & Recommendations
The researchers conclude their findings underscore the importance of SLE as an important public health issue among young females. They advocate for increases in research funding for SLE and state that SLE should be included in the CDC’s selected list of causes of death for its annual ranking. They also have several recommendations for how rheumatologists can help increase awareness, and what patients can do to advocate for themselves.
Drs. Yen and Singh recommend public campaigns to increase awareness among pediatricians and primary care physicians and improve early diagnosis and better management of SLE. To help educate their colleagues in other specialties, rheumatologists can do several things. “First, rheumatologists at academic centers are fortunate to have the opportunity to teach medical students and residents about lupus,” Dr. Yen says.
“Second, rheumatologists can take a more proactive role in educating primary [care] physicians in their community at various venues, including lectures and case presentations at local medical organizations,” Dr. Singh says. He also suggests rheumatology funding agencies assist medical students and residents to allow them to spend time in rheumatology clinics, citing for example the Rheumatology Research Foundation, which offers preceptorships to medical students and residents.
Third, rheumatologists “can help by promoting these opportunities among students and residents via medical education and residency program director’s offices,” Dr. Singh says.
“Fourth, rheumatologists and members of the ACR and other rheumatology organizations can make a case to governments and medical schools for increasing the rheumatology workforce.
“Finally, organizations, such as the ACR and the Foundation, offer grants and initiatives to develop and promote innovative ways of increasing awareness of lupus and other rheumatic diseases.”
Rheumatologists can also encourage their patients to be positive and to maintain hope that treatment will help, Dr. Singh says. This is important because the side effects of lupus treatments are significant. But, he notes, outcomes have been improving over the past 15 years, and it’s important patients know that.
For patients with SLE who are suffering from complications that may seem unrelated to lupus, they and their advocates may need to seek out a rheumatologist to ensure the best care.
“Patients with lupus and their advocates may ask the treating non-rheumatologist physicians if the infection, cardiovascular disease, weight loss, anemia or cancer they may have could be related to lupus and whether contacting their rheumatologist would help their management,” Dr. Singh says.
Because of the increased risk of death among minority women, Dr. Singh adds, “Minority women and their advocates can also cite this study and approach their local county health department to allocate resources to help reduce the high lupus death rate among minority women by hiring an additional rheumatologist or nurses who are trained in lupus care and providing funding for lupus medications.
“Finally, patients and their advocates may contact their representatives in the state or federal government to advocate for the increased funding for lupus research to specifically address the high death rate from lupus in women.”
Kimberly J. Retzlaff is a freelance medical journalist based in Denver.
5 Things You Can Do to Improve the Lupus Outlook
- Educate students and residents about the devastating effects of lupus.
- Take a proactive role to educate primary care physicians in your community, lecturing and presenting case reports at various venues.
- Tell medical students and residents about preceptorship opportunities, and mentor them in clinic situations.
- Get involved in advocacy efforts to ask for lupus research funding; visit the ACR’s Legislative Action Center.
- Ask about ways to get involved in educational efforts via the ACR and other organizations.
- Yen EY, Singh RR. Brief report: Lupus—An unrecognized leading cause of death in young females: A population‐based study using nationwide death certificates, 2000–2015. Arthritis Rheumatol. 2018 Aug;70(8):1251–1255.