OBJECTIVE: To characterize presentation and care pathways of patients with systemic lupus erythematosus (SLE), and delays in access to SLE-specialized care. METHODS: We included patients with incident SLE from the Lupus Midwest Network registry. Time from the first medical encounter for SLE clinical manifestation to access to SLE-specialized care, physician diagnosis, and treatment was estimated. Delays were defined as ≥6 months to access specialized care. We compared SLE manifestations, disease activity (SLEDAI-2k), and SLICC/ACR damage indexes (SDI) between patients with and without delays. Logistic regression models assessed associations with delays. RESULTS: The study included 373 patients with SLE. The median time to access SLE-specialized care was 1.1 months (95% confidence interval [CI] 0.9-1.5); time to diagnosis 30.6 months (95% CI 18.9-48.1), and time to treatment initiation 4.7 months (95% CI 3.9-8.4). Approximately 25% (93/373) of patients experienced delays accessing specialized care, which were associated with fewer SLE manifestations at first SLE-related encounter (<2 SLE domains; 92% vs 72%, P < 0.001). Patients with mucocutaneous or musculoskeletal manifestations were less likely to experience delays, while hematologic (odds ratio [OR] 1.71, 95% CI 1.03-2.84) or antiphospholipid antibodies domains (OR 6.05, 95% CI 2.46-14.88) were associated with delays. Delays were associated with damage at first access to SLE-specialized care (SDI ≥1; 30% vs 7%, P < 0.001). CONCLUSIONS: Patients follow a heterogeneous pathway to receive care. One-fourth of patients experienced delays accessing SLE-specialized care, which was associated with damage. Fewer manifestations, hematologic, or antiphospholipid antibodies were associated with delays.

OBJECTIVES: To assess the prevalence and incidence of multimorbidity and the association with the SLICC/ACR damage index (SDI) among patients with systemic lupus erythematosus (SLE). METHODS: Using prevalent and incident population-based cohorts of patients with SLE and their matched comparators, we assessed 57 chronic conditions. Chronic conditions were categorized as SDI-related or SDI-unrelated. Multimorbidity was defined as the presence of 2+ chronic conditions. Multimorbidity at prevalence and incidence/index was compared between cohorts using logistic regression. Cox models were used to examine development of multimorbidity after SLE incidence. RESULTS: The prevalent cohort included 449 patients with established SLE on January 1, 2015. They were three times more likely to have multimorbidity compared with non-SLE comparators (OR 2.98, 95% CI 2.18-4.11). The incident cohort included 270 patients with new-onset SLE. At SLE incidence, patients with SLE were more likely to have multimorbidity than comparators (OR 2.27, 95% CI 1.59-3.27). After incidence, the risk of developing multimorbidity was 2-fold higher among patients with SLE than comparators (hazard ratio (HR) 2.11, 95% CI 1.59-2.80). Development of multimorbidity was higher in patients with SLE based on SDI-related (HR 2.91, 95% CI 2.17-3.88) and SDI-unrelated conditions (HR 1.73, 95% CI, 1.32-2.26). CONCLUSION: Patients with SLE have a higher burden of multimorbidity, even before the onset of the disease. The risk disparity continues after SLE classification and is also seen in a prevalent SLE cohort. Multimorbidity is driven both by SDI-related and unrelated conditions.

OBJECTIVE: There is little information about the epidemiology and factors associated with opioid therapy in systemic lupus erythematosus (SLE). We aimed to assess the prevalence of opioid therapy and explore factors associated with long-term opioid therapy (LTOT) in patients with SLE. METHODS: Patients with SLE were matched with non-SLE controls in a population-based cohort on January 1, 2015. We captured demographics, manifestations of lupus, comorbidities (fibromyalgia, mood disorders, osteoarthritis, chronic low back pain [CLBP], chronic kidney disease, avascular necrosis, osteoporosis, fragility fractures, and cancer), and the area deprivation index (ADI). Opioid prescription data were used to assess prevalence of LTOT, defined as contiguous prescriptions (gaps of <30 days between prescriptions) and receiving opioid therapy ≥90 days or ≥10 prescriptions before index date. RESULTS: 465 SLE patients and 465 non-SLE controls were included; 13% of SLE patients and 3% of non-SLE controls were receiving opioid therapy (P<0.001), and 11% of SLE patients were on LTOT versus 1% of non-SLE controls. Among SLE patients, acute pericarditis (OR: 3.92; 95% CI: 1.78-8.66), fibromyalgia (OR: 7.78; 95% CI: 3.89-15.55), fragility fractures (OR: 3.72; 95% CI: 1.25-11.07), CLBP (OR: 4.00; 95% CI: 2.13-7.51), and mood disorders (OR: 2.76; 95% CI: 1.47-5.16) were associated with LTOT. We did not find an association between opioid therapy and ADI. CONCLUSION: Patients with SLE have higher LTOT than controls. Among patients with SLE, LTOT was associated with pericarditis and several comorbidities. However, LTOT was not associated with kidney disease despite the limited pain control options in these patients.

OBJECTIVE: The aim of this study was to determine inpatient health care utilization in an incident cohort of patients with systemic lupus erythematosus (SLE) compared with the general population. METHODS: This was a population-based cohort study in the upper Midwest, United States. We included patients fulfilling the European League Against Rheumatism/American College of Rheumatology SLE classification criteria between 1995 and 2018. They were 1:1 age-, sex-, county-matched with individuals without SLE. All hospital admissions and emergency department (ED) visits were electronically retrieved for 1995-2020. Rates for hospital admission, length of stay, readmission, ED visits, and discharge destination were compared between groups. RESULTS: Three hundred forty-one patients with SLE and 341 comparators without SLE were included (mean age, 48.6 years at diagnosis; 79.2% female). Rates of hospitalization for patients with SLE and comparators were 29.8 and 9.9 per 100 person-years, respectively. These differences were present across sexes and age groups. Hospitalization rates were higher in patients with SLE after diagnosis and remained higher than comparators for the first 15 years of the disease. Patients with SLE were more likely than comparators to visit the ED (hazard ratio, 2.71; 95% confidence interval, 2.05-3.59). Readmission rates (32% vs. 21%, p = 0.017) were higher in patients with SLE. Length of stay and discharge destination were similar between both groups. CONCLUSION: Patients with SLE were more likely to be hospitalized and to visit the ED than individuals without SLE, highlighting important inpatient care needs. Increased hospitalization rates were observed in both male and female patients and all age groups.

OBJECTIVES: There is paucity of population-based studies investigating the epidemiology of lupus nephritis (LN) in the US and long-term secular trends of the disease and its outcomes. We aimed to examine the epidemiology of LN in a well-defined eight-county region in the US. METHODS: Patients with incident LN between 1976 and 2018 (1976-2009 Olmsted County, 2010-2018 eight-county region) in Minnesota were identified. Age- and sex-specific incidence rates and point prevalence for four decades, adjusted to the projected 2000 US population, were reported. Standardized mortality ratios (SMR), survival rates, and time to end-stage renal disease (ESRD) were estimated. RESULTS: There were 72 patients with incident LN between 1976-2018. Mean age at diagnosis was 38.4 years (SD 16.24), 76% were female, and 69% non-Hispanic White. Average annual LN incidence between 1976 and 2018 was 1 per 100,000 population (95%CI 0.8-1.3) and highest in the 30-39 age group. Between 1976-1989 and 2000-2018 periods, overall incidence of LN increased from 0.7 to 1.3 per 100,000, but this was not statistically significant. Estimated LN prevalence increased from 16.8 in 1985 to 21.2 per 100,000 in 2015. LN had an SMR of 6.33 (95% CI 3.81-9.89) with no improvement in mortality gap in the last four decades. At 10 years, survival was 70%, and 13% had ESRD. CONCLUSION: The incidence and prevalence of LN in this area increased in the last four decades. LN patients have poor outcomes with high rates of ESRD and mortality rates six times that of the general population. This article is protected by copyright. All rights reserved.

BACKGROUND: Systemic lupus erythematosus (SLE) is a disease that can lead to damage of multiple organs and, along with certain treatments, increase the risk of developing cancer, cardiovascular disease, diabetes, osteoporosis, and infections. Preventive services are particularly important in patients with SLE to mitigate the aforementioned risks. We aimed to evaluate the trends of preventive services utilization in patients with systemic lupus erythematosus, compared with non-SLE population. METHODS: All ≥19-year-old patients in the Lupus Midwest Network (LUMEN) registry, a population-based cohort, with SLE on January 1, 2015, were included and matched (1:1) by sex, age, race, and county to non-SLE comparators. Among both groups, we compared the rates of screenings for breast and cervical cancer, hypertension, hyperlipidemia, diabetes mellitus, and osteoporosis as well as immunizations. RESULTS: We included 440 SLE patients and 430 non-SLE comparators. The probability of breast cancer screening among women with SLE was similar to comparators (hazard ratio [HR] 1.09, 95% CI 0.85-1.39), while cervical cancer screening was lower (HR 0.75, 95% CI 0.58-0.96). Hypertension screening was higher among patients with SLE (HR 1.35, 95% CI 1.13-1.62); however, hyperlipidemia screening was similar to comparators (HR 1.16, 95% CI 0.96-1.41). Diabetes and osteoporosis screenings were more likely to be performed for SLE patients than for comparators (HR 2.46, 95% CI 2.11-2.87; and HR 3.19, 95% CI 2.31-4.41; respectively). Influenza and pneumococcal immunizations were higher among SLE patients (HR 1.31, 95% CI 1.12-1.54; and HR 2.06, 95% CI 1.38-3.09; respectively), while zoster vaccination was similar (HR 1.17, 95% CI 0.81-1.69). CONCLUSIONS: The trends of utilization of preventive services by SLE patients vary according to screening or vaccine compared with the general population. Considering these differences, we demonstrate an opportunity for improvement, particularly in cervical cancer, hyperlipidemia, and osteoporosis screenings and vaccinations.

OBJECTIVE: Systemic lupus erythematosus (SLE) patients are at higher risk of poor outcomes from coronavirus disease 2019 (COVID-19). The vaccination rate among such patients is unknown. We aimed to assess COVID-19 vaccine uptake among SLE patients. METHODS: We included 342 SLE patients from the Lupus Midwest Network and 350 age, sex, race, and county matched comparators. Vaccination uptake for influenza, pneumococcal, and zoster vaccines before pandemic restrictions began (up to February 29, 2020) was assessed. First-dose COVID-19 vaccine uptake was electronically retrieved and manually ascertained (December 15, 2020, to July 31, 2021). Time to COVID-19 vaccination, demographics, lupus manifestations, medications, comorbidity index, area deprivation index, and rurality measures were compared. RESULTS: On July 31, 2021, 83.3% of SLE patients and 85.5% of comparators were vaccinated against COVID-19. The COVID-19 vaccination rates were similar among SLE and comparators (hazard ratio: 0.93; 95% CI: 0.79-1.10). Non-vaccinated SLE patients were more likely to be men (27.3% versus 14.1% vaccinated), younger (mean 54.1 versus 58.8 years in vaccinated), have a shorter SLE duration (median 7.3 versus 10.7 years in vaccinated), and be less frequently vaccinated with influenza and pneumococcal vaccine. CONCLUSION: SLE patients in the Lupus Midwest Network had similar COVID-19 vaccination uptake as matched comparators, most of whom were vaccinated early when the vaccine became available. One in six remain unvaccinated.

OBJECTIVE: To estimate the incidence and time-to-classification of SLE by the 1997 ACR (ACR97) criteria, the SLICC criteria, and the European Alliance of Associations for Rheumatology/ACR (EULAR/ACR) criteria. METHODS: We identified all incident SLE cases from 2000-2018 in the well-defined Olmsted County population. Clinical data included in the ACR97, SLICC and EULAR/ACR criteria were manually abstracted from medical records. All incident cases met at least one of the three classification criteria. Time-to-classification was estimated from the first documented lupus-attributable disease manifestation to the time of criteria fulfilment by each of the three definitions. Annual incidence rates were age or age/sex adjusted to the 2000 US population. RESULTS: Of 139 incident cases there were 126 cases by the EULAR/ACR criteria, corresponding to an age/sex-adjusted incidence of 4.5 per 100 000 population (95% CI: 3.7, 5.2). The age/sex-incidence was higher than that of the SLICC criteria (113 cases; 4.0 per 100 000 [95% CI: 3.3, 4.7], P = 0.020) and the ACR97 (92 cases; 3.3 per 100 000 [95% CI: 2.6, 3.9], P < 0.001). The median time from first disease manifestation to criteria fulfilment was shorter for the EULAR/ACR criteria (29.4 months) than the ACR97 criteria (47.0 months, P < 0.001) and similar to the SLICC criteria (30.6 months, P = 0.83). CONCLUSION: The incidence of SLE was higher by the EULAR/ACR criteria compared with the ACR97 and the SLICC criteria, and the EULAR/ACR criteria classified patients earlier that the ACR97 criteria but similar to the SLICC criteria.

OBJECTIVES: To determine the trends in incidence, prevalence and mortality of systemic lupus erythematosus (SLE) in a US population over four decades. METHODS: We identified all the patients with SLE in Olmsted County, Minnesota who fulfilled the European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) criteria for SLE during 1976-2018. Age-specific and sex-specific incidence and prevalence dates were adjusted to the standard 2000 projected US population. The EULAR/ACR score was used as a proxy for disease severity. Standardised mortality ratio (SMR) was estimated. RESULTS: There were 188 incident SLE cases in 1976-2018 (mean age 46.3±SD 16.9; 83% women). Overall age-adjusted and sex-adjusted annual SLE incidence per 100 000 population was 4.77 (95% CI 4.09 to 5.46). Incidence was higher in women (7.58) than men (1.89). The incidence rate increased from 3.32 during 1976-1988 to 6.44 during 2009-2018. Incidence rates were higher among the racial and ethnic minority populations than non-Hispanic whites. The EULAR/ACR score did not change significantly over time. Overall prevalence increased from 30.6 in 1985 to 97.4 in 2015. During the study period, there was no improvement in SMR over time (p=0.31). CONCLUSIONS: The incidence and prevalence of SLE are increasing in this US population. The increase in incidence may be at least partially explained by the rising ethnic/racial diversity of the population. There was no evidence that the severity of SLE has changed over time. The survival gap between SLE and the general population remains unchanged. As the US population grows more diverse, we might continue to see an increase in the incidence of SLE.

OBJECTIVE: To characterize the incidence and prevalence of childhood onset systemic lupus erythematosus (cSLE), and to estimate the proportion of patients who are diagnosed with SLE during childhood. METHODS: A cohort of patients with incident cSLE in 1976 to 2018 from a US 8-county region were identified based on comprehensive medical record review. All cases met the European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) or ACR 97 criteria at or before age 18. Incidence rates were estimated using Poisson methods. We estimated the cSLE point prevalence for 1/1/2015. Results were sex/age-adjusted to the US 2000 population. Among all the SLE patients living in the 8-county region in 1/1/2015, the proportion of patients diagnosed at ≤18 years was estimated. RESULTS: A total of 13 children were diagnosed with cSLE during the study period (EULAR/ACR definition, mean age at diagnosis 15.1 years; 85% female, 69% White). cSLE overall adjusted incidence rate was 0.7 (95% CI, 0.2-1.1) per 100,000 children. Incidence rate in girls was 1.2 (95% CI, 0.5-1.9) per 100,000, while in boys it was 0.2 (95% CI, 0.0-0.5) per 100,000. Adjusted prevalence of cSLE was 1.1 (95% CI,0.0-3.1) per 100,000 children. The proportion of patients with SLE diagnosed as children was 9% (95% CI, 6-13%). CONCLUSIONS: In this population-based study, both the incidence and prevalence rates of cSLE were ~ 1 per 100,000 children. One in ten adults with SLE were diagnosed in childhood. More studies are needed to further characterize the epidemiology of cSLE in minorities.