Steady decrease in systemic sclerosis mortality rates at younger ages over the past five decades.

OBJECTIVE: We aimed to assess SSc mortality by age in the general population over the past five decades. METHODS: This is a population-based study using a national mortality database and the census data for all US residents. We calculated the proportions of deaths for SSc and for all other causes (non-SSc) by age, and calculated age-standardized mortality rates (ASMRs) for SSc and non-SSc, and the ratio of SSc-ASMR to non-SSc-ASMR by age groups for each year from 1968 through 2015. We performed joinpoint regression to estimate the average annual percent change (AAPC) for each of these parameters. RESULTS: SSc was recorded as the underlying cause of death in 5457 decedents aged ≤44 years, 18 395 aged 45-64, and 22 946 aged ≥65 from 1968 through 2015. At ages ≤44, the proportion of annual deaths decreased more for SSc than for non-SSc: AAPC, -2.2% (95% CI, -2.4% to -2.0%) for SSc vs -1.5% (-1.9% to -1.1%) for non-SSc. Consistently, SSc-ASMR decreased from 1.0 (95% CI, 0.8-1.2) in 1968 to 0.4 (0.3-0.5) per million persons in 2015, a cumulative decrease of 60% at an AAPC of -1.9% (95% CI, -2.5% to -1.2%) at ages ≤44. The SSc-ASMR:non-SSc-ASMR ratio also decreased [cumulative -20%; AAPC -0.3% (95% CI, -1.15% to 0.55%)] in the ≤44-years group. In contrast, those aged ≥65 experienced a steep increase in the SSc-ASMR [cumulative 187.0%; AAPC 2.0% (95% CI, 1.8-2.2)] and the SSc-ASMR:non-SSc-ASMR ratio [cumulative 395.4%; AAPC 3.3% (95% CI, 2.9-3.7)]. CONCLUSION: Mortality for SSc has steadily decreased at younger ages over the past five decades.

46-Year Trends in Systemic Lupus Erythematosus Mortality in the United States, 1968 to 2013: A Nationwide Population-Based Study.

BACKGROUND: No large population-based studies have been done on systemic lupus erythematosus (SLE) mortality trends in the United States. OBJECTIVE: To identify secular trends and population characteristics associated with SLE mortality. DESIGN: Population-based study using a national mortality database and census data. SETTING: United States. PARTICIPANTS: All U.S. residents, 1968 through 2013. MEASUREMENTS: Joinpoint trend analysis of annual age-standardized mortality rates (ASMRs) for SLE and non-SLE causes by sex, race/ethnicity, and geographic region; multiple logistic regression analysis to determine independent associations of demographic variables and period with SLE mortality. RESULTS: There were 50 249 SLE deaths and 100 851 288 non-SLE deaths from 1968 through 2013. Over this period, the SLE ASMR decreased less than the non-SLE ASMR, with a 34.6% cumulative increase in the ratio of the former to the latter. The non-SLE ASMR decreased every year starting in 1968, whereas the SLE ASMR decreased between 1968 and 1975, increased between 1975 and 1999, and decreased thereafter. Similar patterns were seen in both sexes, among black persons, and in the South. However, statistically significant increases in the SLE ASMR did not occur among white persons over the 46-year period. Females, black persons, and residents of the South had higher SLE ASMRs and larger cumulative increases in the ratio of the SLE to the non-SLE ASMR (31.4%, 62.5%, and 58.6%, respectively) than males, other racial/ethnic groups, and residents of other regions, respectively. Multiple logistic regression showed independent associations of sex, race, and region with SLE mortality risk and revealed significant racial/ethnic differences in associations of SLE mortality with sex and region. LIMITATIONS: Underreporting of SLE on death certificates may have resulted in underestimates of SLE ASMRs. Accuracy of coding on death certificates is difficult to ascertain. CONCLUSION: Rates of SLE mortality have decreased since 1968 but remain high relative to non-SLE mortality, and significant sex, racial, and regional disparities persist. PRIMARY FUNDING SOURCE: None.

Worsening premature death burden gap from systemic sclerosis in men and black persons: A US nationwide population-based study.

Objective: Male sex and black race incur poor prognosis in systemic sclerosis (SSc). There is no nationwide population-based assessment of premature SSc death burden by sex and race. Methods: This is a population-based study comprising all recorded SSc deaths across the United States. We constructed histograms depicting the number of SSc deaths for each age by sex and race, and calculated the cumulative percent death at each age and the median age of death. We determined the odds ratios for the risk of premature death from SSc by sex and race. We then calculated the percent of total SSc deaths for different age groups by sex and race from 1970 to 2015. We performed chi-square test with Yates's correction and quantified the odds ratio (OR) with 95% confidence interval (CI). Results: The median age of SSc death was 63 years in males versus 68 years in females, and 57 years in blacks versus 70 years in whites. The odds for SSc death before 65 years age was 1.8 (95% CI, 1.6-2.0) for males compared with females and 5.1 (95% CI, 4.4-6.0) for blacks compared with whites. The higher odds for premature death in males than in females was similar for both races. Differences in the proportions of premature deaths from 1970 to 2015 increased between males and females (-5% to 17%) and between blacks and whites (14% to 36%). Conclusion: Males and black persons die of SSc at younger ages. The worsening premature death burden gap between the two sexes and races over the last five decades is troublesome.

Consensus Treatment Plans for Severe Pediatric Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

OBJECTIVE: There is no standardized approach to the treatment of pediatric antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Because of the rarity of pediatric AAV, randomized trials have not been feasible. The present study of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) was undertaken to establish consensus treatment plans (CTPs) for severe pediatric AAV to enable the future study of comparative effectiveness and safety. METHODS: A workgroup of CARRA members (rheumatologists and nephrologists) formed the AAV Workgroup. This group performed a literature review on existing evidence-based treatments and guidelines for the management of AAV. They determined that the target population for CTP development was patients <18 years of age with new-onset granulomatosis with polyangiitis (GPA), microscopic polyangiitis, or renal-limited AAV (eosinophilic GPA was excluded), with presentation confined to those with severe disease (i.e., organ- or life-threatening). Face-to-face consensus conferences employed nominal group techniques to identify treatment strategies for remission induction and remission maintenance, data elements to be systematically collected, and outcomes to be measured over time. RESULTS: The pediatric AAV Workgroup developed 2 CTPs for each of the remission induction and remission maintenance of severe AAV. A glucocorticoid-weaning regimen for induction and maintenance, a core data set, and outcome measures were also defined. A random sample of CARRA membership voted acceptance of the CTPs for remission induction and remission maintenance, with a 94% (75 of 80) and 98% (78 of 80) approval rate, respectively. CONCLUSION: Consensus methodology established standardized CTPs for treating severe pediatric AAV. These CTPs were in principle accepted by CARRA-wide membership for the evaluation of pragmatic comparative effectiveness in a long-term registry.

Trends in Systemic Sclerosis Mortality Over Forty-Eight Years, 1968-2015: A US Population-Based Study.

OBJECTIVE: To identify secular trends associated with systemic sclerosis (SSc) mortality over a 48-year period. METHODS: Using national mortality data compiled by the Centers for Disease Control and Prevention's Wide-Ranging Online Data for Epidemiologic Research, and population data from the US Census Bureau, we calculated an age-standardized mortality rate (ASMR) for SSc and non-SSc (all other causes), and we also calculated the ratio of the SSc ASMR to the non-SSc ASMR for each year from 1968 to 2015. We then used a joinpoint regression model to evaluate mortality trends overall and by sex and race. RESULTS: From 1968 to 2015, there were 46,798 deaths with SSc recorded as the "underlying" cause of death and 106,058,839 non-SSc deaths. There were an additional 9,063 deaths with SSc recorded as a "contributing" cause of death from 1999 to 2015. Whereas the non-SSc ASMR decreased throughout the 48-year time period, the SSc ASMR increased from 1968 to 2000, followed by decreases each year from 2001 to 2015. The SSc ASMR also decreased for deaths where SSc was a contributing cause from 1999 to 2015. Women and Black persons had higher SSc ASMRs and SSc ASMR to non-SSc ASMR ratios than men and White persons, respectively. Additionally, SSc ASMRs and SSc ASMR to non-SSc ASMR ratios increased at higher rates in women and White persons than in men and Black persons, respectively, during the initial three decades. CONCLUSION: Mortality attributable to SSc increased from 1968 to 2000, followed by a steady decline from 2001 to 2015. However, SSc mortality relative to non-SSc mortality remains high. SSc mortality has disproportionately changed by sex and race over the 48-year period assessed in the present study.

Brief Report: Lupus-An Unrecognized Leading Cause of Death in Young Females: A Population-Based Study Using Nationwide Death Certificates, 2000-2015.

OBJECTIVE: Mortality statistics from the Centers for Disease Control and Prevention (CDC) are used for planning health care policy and allocating resources. The CDC uses these data to compile its annual ranking of leading causes of death based on a selected list of 113 causes. Systemic lupus erythematosus (SLE) is not included on this list. Since the ranking is a useful tool for assessing the relative burden of cause-specific mortality, this study was undertaken to rank SLE deaths among the CDC's leading causes of death to see whether SLE is a significant cause of death among females. METHODS: Death counts for the female population of the US were obtained from the CDC's Wide-ranging Online Data for Epidemiologic Research database and then grouped by age and race/ethnicity. Data on the leading causes of death were obtained from the Web-based Injury Statistics Query and Reporting System database. RESULTS: During 2000-2015, there were 28,411 deaths of females with SLE recorded as an underlying or contributing cause of death. SLE ranked among the top 20 leading causes of death in females between 5 and 64 years of age. SLE ranked tenth among those ages 15-24 years, fourteenth among those ages 25-34 years and 35-44 years, and fifteenth among those ages 10-14 years. For African American and Hispanic females, SLE ranked fifth among those ages 15-24 years, sixth among those ages 25-34 years, and eighth or ninth among those ages 35-44 years, after the 3 common external injury causes of death were excluded from the analysis. CONCLUSION: SLE is among the leading causes of death in young females, underscoring its impact as an important public health issue.