Lupus Deaths in Two Georgia Counties: Black Patients Died Younger and Sooner

Systemic lupus erythematosus (SLE) is a long-lasting autoimmune disease that can affect the whole body. Its symptoms are often vague, which can delay diagnosis, and it falls hardest on women and minorities. Black patients tend to develop it younger and to have more severe disease. Survival has improved — five-year survival rose from 50% in 1955 to about 90% in the 2000s, largely thanks to better management — but people with lupus still die early, often from severe disease, infections or cardiovascular disease.

Counting those deaths is not straightforward. Death certificates are known to miss many of them: they are estimated to capture only 40%–60% of lupus cases. A CDC report took a different approach, starting from a registry of people known to have lupus and following them forward.

The registry

The Georgia Lupus Registry was set up to find every resident with lupus in two counties of metropolitan Atlanta, Fulton and DeKalb, which have large Black and white populations. Investigators could collect health information without each patient's written consent under the public health surveillance exemption to the HIPAA Privacy Rule. That let them find every potential case, confirm whether it met the definition, and avoid counting the same patient twice when they had been seen in several places. The information was stored securely, seen only by authorized research staff, and de-identified wherever possible.

Cases came from hospitals, rheumatologists and nephrology and dermatology groups in and around the two counties, whose billing records were searched for lupus and related conditions, as well as from laboratories and other population databases.

A patient counted as having lupus if they met at least four of the American College of Rheumatology's 11 classification criteria (1997 update of the 1982 revision), or three of them together with a lupus diagnosis documented by their board-certified rheumatologist. The 11 criteria are malar rash, discoid rash, sensitivity to light, mouth ulcers, arthritis, serositis, kidney disorder, neurologic disorder, blood disorder, immunologic disorder and antinuclear antibody.

Two groups were followed:

• 1,353 prevalent cases — people living with lupus in 2002.
• 336 incident cases — people newly diagnosed during 2002–2004. They were demographically similar to the first group (87%–90% female, 74%–76% Black, 23% white) but were older at diagnosis, a mean of 40.6 years against 34.6.

Every patient was matched against the National Death Index through 2016. Causes of death were available but not analyzed, because death records are unreliable about whether lupus was involved.

What the registry found

By 2016, 401 people in the prevalent group and 97 in the incident group had died.

Deaths compared with the general population. Among the Black and white prevalent patients, the report compared deaths with the number expected in the general population of the two counties for people of the same age, sex and race:

Black women with lupus were three times as likely to die as Black women in the general population. The incident group had too few deaths for this comparison.

Black patients died sooner. Cumulative mortality was significantly higher for Black than for white patients in both groups. Among the newly diagnosed, no white patient died until five years after diagnosis, while deaths among Black patients were higher from the moment of diagnosis and stayed higher. White patients reached 9% cumulative mortality after 10 years; Black patients reached the same level after 2.

And younger. The mean age at death for Black patients was 51.8 years in the incident group and 52.3 in the prevalent group, against 64.4 and 65.0 for white patients — about 13 years younger.

There were no significant differences between men and women.

Cumulative mortality of patients diagnosed with lupus during 2002–2004, Black and white, Georgia Lupus Registry, 2002–2016. CDC, MMWR.

Cumulative mortality of patients with lupus in 2002, Black and white, Georgia Lupus Registry, 2002–2016. CDC, MMWR.

What it means

Lupus remains deadly despite growing awareness and better treatment, and the highest ratio was among Black women. The disparity weighs even more because lupus is three times as common in Black people as in white people. Earlier studies — a 2002 study, and a nationwide study of death certificates from 1968 through 2013 — also found the highest burden among women and Black people, but both relied on death certificates to identify lupus.

The authors noted four limitations: race was mostly assigned from the physician's notes and may not match how patients identify themselves; some cases may have been missed; diagnosis may vary between rheumatologists, and undiagnosed cases were not sought; and the results may not apply outside the two counties. The registry's strengths were that it identified nearly all confirmed cases in the area and followed them for a long time, finding more deaths than death certificates alone would.

The authors suggested focusing on causes of death that can be reversed. CDC supports efforts to encourage early detection, diagnosis and treatment and to build patients' self-management skills, including work by the Lupus Foundation of America and the American College of Rheumatology to help providers diagnose lupus accurately. The first National Public Health Agenda for Lupus sets out a public health plan for the disease.

Sources

• Lim SS, Helmick CG, Bao G, Hootman J, Bayakly R, Gordon C, Drenkard C. "Racial Disparities in Mortality Associated with Systemic Lupus Erythematosus — Fulton and DeKalb Counties, Georgia, 2002–2016." MMWR 68(18). CDC. The report describes the mortality ratios as two to three times, and as 2.3–3.3 times, higher than expected, while its table ranges from 2.36 to 3.38; the table's figures are given here. The table covers 1,335 patients and 400 deaths, after 18 people who were neither Black nor white, one of whom died, were left out.