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Objective Epidemiological data for MCTD are limited. Leveraging data from the Manhattan Lupus Surveillance Program (MLSP), a racially/ethnically diverse population-based registry of cases with SLE and related diseases including MCTD, we provide estimates of the prevalence and incidence of MCTD. Methods MLSP cases were identified from rheumatologists, hospitals and population databases using a variety of International Classification of Diseases, Ninth Revision codes. MCTD was defined as one of the following: fulfilment of our modified Alarcon-Segovia and Kahn criteria, which required a positive RNP antibody and the presence of synovitis, myositis and RP; a diagnosis of MCTD and no other diagnosis of another CTD; and a diagnosis of MCTD regardless of another CTD diagnosis. Results Overall, 258 (7.7%) cases met a definition of MCTD. Using our modified Alarcon-Segovia and Kahn criteria for MCTD, the age-adjusted prevalence was 1.28 (95% CI 0.72, 2.09) per 100 000. Using our definition of a diagnosis of MCTD and no other diagnosis of another CTD yielded an age-adjusted prevalence and incidence of MCTD of 2.98 (95% CI 2.10, 4.11) per 100 000 and 0.39 (95% CI 0.22, 0.64) per 100 000, respectively. The age-adjusted prevalence and incidence were highest using a diagnosis of MCTD regardless of other CTD diagnoses and were 16.22 (95% CI 14.00, 18.43) per 100 000 and 1.90 (95% CI 1.49, 2.39) per 100 000, respectively. Conclusions The MLSP provided estimates for the prevalence and incidence of MCTD in a diverse population. The variation in estimates using different case definitions is reflective of the challenge of defining MCTD in epidemiologic studies. [ABSTRACT FROM AUTHOR]

Objective: To develop an evidence-based guideline on contraception, assisted reproductive technologies (ART), fertility preservation with gonadotoxic therapy, use of menopausal hormone replacement therapy (HRT), pregnancy assessment and management, and medication use in patients with rheumatic and musculoskeletal disease (RMD).Methods: We conducted a systematic review of evidence relating to contraception, ART, fertility preservation, HRT, pregnancy and lactation, and medication use in RMD populations, using Grading of Recommendations Assessment, Development and Evaluation methodology to rate the quality of evidence and a group consensus process to determine final recommendations and grade their strength (conditional or strong). Good practice statements were agreed upon when indirect evidence was sufficiently compelling that a formal vote was unnecessary.Results: This American College of Rheumatology guideline provides 12 ungraded good practice statements and 131 graded recommendations for reproductive health care in RMD patients. These recommendations are intended to guide care for all patients with RMD, except where indicated as being specific for patients with systemic lupus erythematosus, those positive for antiphospholipid antibody, and/or those positive for anti-Ro/SSA and/or anti-La/SSB antibodies. Recommendations and good practice statements support several guiding principles: use of safe and effective contraception to prevent unplanned pregnancy, pre-pregnancy counseling to encourage conception during periods of disease quiescence and while receiving pregnancy-compatible medications, and ongoing physician-patient discussion with obstetrics/gynecology collaboration for all reproductive health issues, given the overall low level of available evidence that relates specifically to RMD.Conclusion: This guideline provides evidence-based recommendations developed and reviewed by panels of experts and RMD patients. Many recommendations are conditional, reflecting a lack of data or low-level data. We intend that this guideline be used to inform a shared decision-making process between patients and their physicians on issues related to reproductive health that incorporates patients' values, preferences, and comorbidities. [ABSTRACT FROM AUTHOR]

Background: Although pregnancy in systemic lupus erythematosus (SLE) carries a high risk for mother and fetus, outcomes may be improving.Objective: To assess nationwide trends and disparities in maternal and fetal complications among pregnant women with SLE.Design: Retrospective cohort study.Setting: United States, 1998 to 2015.Patients: Adult pregnant women with and without SLE who had hospitalizations recorded in the National Inpatient Sample (NIS) database.Measurements: Outcome measures were in-hospital maternal mortality, fetal mortality, preeclampsia or eclampsia, caesarean sections, non-delivery-related admissions, and length of stay. To assess whether trends in outcomes over time differed between patients with SLE and those without SLE, logistic or linear regression with an interaction term between year and SLE (yes or no) was used. Nationwide population estimates incorporating sampling and poststratification weights were obtained.Results: An estimated 93 820 pregnant women with SLE and 78 045 054 without SLE were hospitalized in the United States from 1998 through 2015. Outcomes improved during those 18 years. In-hospital maternal deaths (per 100 000 admissions) declined among patients with as well as those without SLE (442 vs. 13 for 1998 to 2000 and <50 vs. 10 for 2013 to 2015), although the decrease was greater in women with SLE (difference in trends, P < 0.002). The percentage of patients with SLE in all pregnancy-related, as well as delivery-related, admissions increased significantly.Limitations: The sample for this analysis was identified by using diagnostic codes; detailed information on hospital-specific trends, SLE disease activity, and medications was not available. Race trends could not be analyzed. Given that NIS uses weighted estimates, the incidence of outcomes reported may not be exact.Conclusion: In this large study examining SLE and non-SLE pregnancies over 18 years, in-hospital maternal mortality and overall outcomes improved markedly, particularly among women with SLE. However, improvement is still needed, because SLE pregnancy risks remain high.Primary Funding Source: None. [ABSTRACT FROM AUTHOR]

Objective: We examined rates of adverse pregnancy outcomes (APO) by race/ethnicity among women with systemic lupus erythematosus (SLE), with and without antiphospholipid antibodies (aPL), and whether socioeconomic status (SES) accounted for differences.Methods: Data were from the PROMISSE (Predictors of Pregnancy Outcome: Biomarkers in Antiphospholipid Antibody Syndrome and Systemic Lupus Erythematosus) study, a multicenter study that enrolled 346 patients with SLE and 62 patients with SLE and aPL (50% white, 20% African American, 17% Hispanic, 12% Asian/Pacific Islander). Measures of SES were educational attainment, median community income, and community education. Logistic regression analyses were conducted to determine odds of APO for each racial/ethnic group, controlling first for age and clinical variables, and then for SES.Results: The frequency of APO in white women with SLE, with and without aPL, was 29% and 11%, respectively. For African American and Hispanic women it was approximately 2-fold greater. In African American women with SLE alone, adjustment for clinical variables attenuated the odds ratio (OR) from 2.7 (95% confidence interval [95% CI] 1.3-5.5) to 2.3 (95% CI 1.1-5.1), and after additional adjustment for SES, there were no longer significant differences in APO compared to whites. In contrast, in SLE patients with aPL, whites, African Americans, and Hispanics had markedly higher risks of APO compared to white SLE patients without aPL (OR 3.5 [95% CI 1.4-7.7], OR 12.4 [95% CI 1.9-79.8], and OR 10.4 [95% CI 2.5-42.4], respectively), which were not accounted for by clinical or SES covariates.Conclusion: This finding suggests that for African American women with SLE without aPL, SES factors are key contributors to disparities in APO, despite monthly care from experts, whereas other factors contribute to disparities in SLE with aPL. [ABSTRACT FROM AUTHOR]

Objective To study the effect of the innate cytokine macrophage migration inhibitory factor (MIF) on the susceptibility and severity of systemic lupus erythematosus (SLE) in a multinational population of 1,369 Caucasian and African American patients. Methods Two functional polymorphisms in the MIF gene, a −794 CATT5-8 microsatellite repeat ( rs5844572) and a −173 G/C single-nucleotide polymorphism ( rs755622), were assessed for association with SLE in 3,195 patients and healthy controls. We also measured MIF plasma levels in relation to genotypes and clinical phenotypes, and assessed Toll-like receptor 7 (TLR-7)-stimulated MIF production in vitro. Results Both Caucasians and African Americans with the high-expression MIF haplotype −794 CATT7/−173*C had a lower incidence of SLE (in Caucasians, odds ratio [OR] 0.63, 95% confidence interval [95% CI] 0.53-0.89, P = 0.001; in African Americans, OR 0.46, 95% CI 0.23-0.95, P = 0.012). In contrast, among patients with established SLE, reduced frequencies of low-expression MIF genotypes (−794 CATT5) were observed in those with nephritis, those with serositis, and those with central nervous system (CNS) involvement when compared to patients without end-organ involvement ( P = 0.023, P = 0.005, and P = 0.04, respectively). Plasma MIF levels and TLR-7-stimulated MIF production in vitro reflected the underlying MIF genotype of the studied groups. Conclusion These findings suggest that MIF, which has both proinflammatory properties and macrophage and B cell survival functions, exerts a dual influence on the immunopathogenesis of SLE. High-expression MIF genotypes are associated with a reduced susceptibility to SLE and may contribute to an enhanced clearance of infectious pathogens. Once SLE develops, however, low-expression MIF genotypes may protect from ensuing inflammatory end-organ damage. [ABSTRACT FROM AUTHOR]

In the United States, between 1 and 3% of women suffer recurrent miscarriages; 50-70% of all conceptions fail. [1,2] Although in the majority of affected women the cause of recurrent miscarriages is unknown, an immune mechanism involving the inappropriate and subsequently injurious recognition of the conceptus by the mother's immune system has been proposed. Murine models have recently been developed that are relevant to this issue. We and others have identified a novel role for complement as an early effector in the pathway leading to pregnancy loss associated with placental inflammation. Indeed, it appears that inhibition of complement activation is an absolute requirement for normal pregnancy, and that in the antiphosphospholid syndrome overwhelming activation of complement triggered by antibodies (Ab) deposited in placenta leads to fetal injury. Identification of complement activation as a mediator of pregnancy loss and definition of the complement components necessary to trigger such injury is likely to lead to a better understanding of its pathogenesis and to new and improved treatments. [ABSTRACT FROM AUTHOR]

Objective: Using the Manhattan Lupus Surveillance Program, a multiracial/ethnic population-based registry, we aimed to compare 3 commonly used classification criteria for systemic lupus erythematosus (SLE) to identify unique cases and determine the incidence and prevalence of SLE using the EULAR/American College of Rheumatology (ACR) criteria., Methods: SLE cases were defined as fulfilling the 1997 ACR, the Systemic Lupus International Collaborating Clinics (SLICC), or the EULAR/ACR classification criteria. We quantified the number of cases uniquely associated with each and the number fulfilling all 3 criteria. Prevalence and incidence using the EULAR/ACR classification criteria and associated 95% confidence intervals (95% CIs) were calculated., Results: A total of 1,497 cases fulfilled at least 1 of the 3 classification criteria, with 1,008 (67.3%) meeting all 3 classifications, 138 (9.2%) fulfilling only the SLICC criteria, 35 (2.3%) fulfilling only the 1997 ACR criteria, and 34 (2.3%) uniquely fulfilling the EULAR/ACR criteria. Patients solely satisfying the EULAR/ACR criteria had <4 manifestations. The majority classified only by the 1997 ACR criteria did not meet any of the defined immunologic criteria. Patients fulfilling only the SLICC criteria did so based on the presence of features unique to this system. Using the EULAR/ACR classification criteria, age-adjusted overall prevalence and incidence rates of SLE in Manhattan were 59.6 (95% CI 55.9-63.4) and 4.9 (95% CI 4.3-5.5) per 100,000 population, with age-adjusted prevalence and incidence rates highest among non-Hispanic Black female patients., Conclusion: Applying the 3 commonly used classification criteria to a population-based registry identified patients with SLE fulfilling only 1 validated definition. The most recently developed EULAR/ACR classification criteria revealed prevalence and incidence estimates similar to those previously established for the ACR and SLICC classification schemes., (© 2022 American College of Rheumatology.)

Background: Studies of proliferative lupus nephritis (PLN) suggest that African-Americans have a poorer prognosis than Whites. However, no study has simultaneously examined socio-economic status. We studied rates of progression of PLN among a tri-ethnic population with respect to socio-economic status and race/ethnicity., Methods: A retrospective cohort study was carried out using individual and census-based neighbourhood data. Consecutive patients in urban tertiary care centres with biopsy-proven PLN were studied. The main outcome was time to doubling of serum creatinine., Results: Among 128 patients with PLN, the percentage of patients who did not double their serum creatinine at 5 years was 67.0% (+/-4.8%) and at 10 years was 58.9% (+/-5.7%). In bivariate analyses, residence in a poor neighbourhood was positively associated with progression (P = 0.03), as was African-American and Hispanic race/ethnicity (P = 0.01). Residence in a poor neighbourhood remained associated with progression of disease after adjustment for age, sex, creatinine, hypertension, cyclophosphamide treatment and race/ethnicity [relative risk (RR) 3.5, 95% confidence interval (CI) 1.2-11, P = 0.03]. After adjustment for poverty and insurance, the RR for African-American race/ethnicity was reduced from 3.5 to 2.7 and was not statistically associated with progression of disease in the full model (P = 0.10). A similar reduction in RR from 5.5 to 3.6 was seen for Hispanic race/ethnicity, but this retained statistical significance (P = 0.03)., Conclusions: Poverty is an important risk factor for progression of PLN, independent of race/ethnicity. Hispanics have an elevated risk similar to or greater than African-Americans. Given these findings, some of the poorer prognosis of African-American patients with PLN may result from socio-economic rather than biological or genetic factors.