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Objective: Damage accrual in SLE has been previously shown to be an independent predictor of mortality. We sought to discern which SLICC Damage Index (SDI) domains are the most important predictors of survival in SLE., Methods: SLE patients (ACR criteria), age > or =16 years, disease duration < or =5 years at enrolment, of African-American, Hispanic or Caucasian ethnicity were studied. Disease activity was assessed using the SLAM-Revised (SLAM-R) at diagnosis. Damage was ascertained using the SDI at the last visit. The SDI domains associated with time to death (and interaction terms) were examined by univariable and multivariable Cox proportional hazards regression analyses; those significant in the multivariable analyses were added to the final two models (with and without poverty) that included other variables known to be associated with shorter survival., Results: A total of 635 SLE patients were studied of whom 97 (15.3%) have died over a mean (s.d.) total disease duration of 5.7 (3.7) years. Patients were predominantly women [570 (89.8%)]; their mean (s.d.) age was 36.5 (12.6) years; 126 (19.8%) had developed renal damage, 62 (9.3%) cardiovascular, 48 (7.8%) pulmonary and 34 (5.4%) peripheral vascular damage. When excluding poverty from the multivariable model, the renal domain of the SDI was independently associated with a shorter time to death (hazard ratio = 1.65; 95% CI 1.03, 2.66)., Conclusions: The renal domain of the damage index is associated with a shorter time to death when poverty, a strong predictor of this outcome, is removed from the model. Preventing renal damage in lupus patients has long-term prognostic implications.

Objective: To examine health disparities as a function of ethnicity using data from LUpus in MInorities, NAture versus nurture (LUMINA), a longitudinal study of patients with systemic lupus erythematosus (SLE); to build an explanatory model of how ethnic disparities occur in this setting; and to suggest appropriate interventions., Methods: LUMINA patients (meeting American College of Rheumatology criteria for SLE) ages >/=16 years of African American, Hispanic (from Texas), Hispanic (from Puerto Rico), or Caucasian ethnicity were studied. In addition to examining the basic features of the cohort, we examined, by univariable and multivariable analyses, the factors associated with disease activity, damage accrual, lupus nephritis, and mortality. An empiric model based on the data presented (and the literature reviewed) was derived to explain the disparities observed., Results: There were substantial differences in the socioeconomic/demographic, clinical, and genetic features among patients from the different ethnic groups, with Texan Hispanic and African American patients exhibiting overall a lower socioeconomic status, different genetic associations, more serious disease at a younger age, and worse intermediate and final outcomes than the Caucasian and Puerto Rican Hispanic patients. A model of disease outcome as a function of the disparities observed was created., Conclusion: Ethnic disparities occur in SLE. Environmental, socioeconomic/demographic, psychosocial, genetic, and clinical factors play an important role as determinants of the ethnic differences observed. Measures aimed at eliminating these disparities are suggested while further research is conducted to elucidate the basis of these disparities and their changes at the societal level and to eliminate the gap between the rich and the poor.

Background: Renal involvement is a serious manifestation of systemic lupus erythematosus (SLE); it may portend a poor prognosis as it may lead to end-stage renal disease (ESRD). The purpose of this study was to determine the factors predicting the development of renal involvement and its progression to ESRD in a multi-ethnic SLE cohort (PROFILE)., Methods and Findings: PROFILE includes SLE patients from five different United States institutions. We examined at baseline the socioeconomic-demographic, clinical, and genetic variables associated with the development of renal involvement and its progression to ESRD by univariable and multivariable Cox proportional hazards regression analyses. Analyses of onset of renal involvement included only patients with renal involvement after SLE diagnosis (n = 229). Analyses of ESRD included all patients, regardless of whether renal involvement occurred before, at, or after SLE diagnosis (34 of 438 patients). In addition, we performed a multivariable logistic regression analysis of the variables associated with the development of renal involvement at any time during the course of SLE. In the time-dependent multivariable analysis, patients developing renal involvement were more likely to have more American College of Rheumatology criteria for SLE, and to be younger, hypertensive, and of African-American or Hispanic (from Texas) ethnicity. Alternative regression models were consistent with these results. In addition to greater accrued disease damage (renal damage excluded), younger age, and Hispanic ethnicity (from Texas), homozygosity for the valine allele of FcgammaRIIIa (FCGR3A*GG) was a significant predictor of ESRD. Results from the multivariable logistic regression model that included all cases of renal involvement were consistent with those from the Cox model., Conclusions: Fcgamma receptor genotype is a risk factor for progression of renal disease to ESRD. Since the frequency distribution of FCGR3A alleles does not vary significantly among the ethnic groups studied, the additional factors underlying the ethnic disparities in renal disease progression remain to be elucidated.

Objective: To determine if different categories of erythrocyte sedimentation rate (ESR) elevation are associated with disease activity and/or damage in systemic lupus erythematosus (SLE)., Methods: We studied 2317 study visits in 553 SLE patients (> or = 4 American College of Rheumatology criteria, < or = 5 years' disease duration at enrollment) from a multiethnic (Hispanic, African American, and Caucasian) longitudinal study of outcome. A study visit was done every 6 months for the first year and annually thereafter. Erythrocyte sedimentation rate (ESR) was measured using the Westergren method; results were expressed in 4 categories: < 25 (normal), 25-50 (mild elevation), 51-75 (moderate elevation), and > 75 (marked elevation) mm/h. Anti-dsDNA antibodies were measured at enrollment with the Crithidia luciliae assay. Disease activity was assessed with the Systemic Lupus Activity Measure (SLAM) and the Physician's Global Assessment (PGA). Because ESR is one of the measures evaluated in the SLAM, it was excluded from the total SLAM score. Disease damage was assessed with the Systemic Lupus International Collaborating Clinics damage index (SDI). The relationship between the SLAM (total and PGA) and SDI scores (at baseline and for all visits) and anti-dsDNA antibodies (at enrollment) with ESR was examined by univariable and generalized estimating equation (GEE) regression analyses. Ethnicity, age, and sex were entered in all regression models., Results: The cohort consisted of 89.7% women with mean age 36.8 (SD 12.6) years and disease duration 4.6 (SD 3.2) years. GEE analyses showed that increasing levels of ESR and anti-dsDNA antibody positivity were independently associated with SLAM and PGA scores, at enrollment and for all visits. Overall, the associations of ESR with SLAM and PGA scores were stronger than for the presence of anti-dsDNA antibodies. At baseline, there was no relationship of ESR elevation or anti-dsDNA positivity with SDI scores. However, when all visits were studied, moderate and marked elevations of ESR were independently associated with SDI scores., Conclusion: Mild, moderate, and marked ESR elevations are strongly associated with disease activity in SLE. Moderate and marked ESR elevations are also associated with damage accrual. These associations are stronger than those for the presence of anti-dsDNA antibodies. Our data suggest that ESR could be used to assess disease activity and predict organ/system damage in a relatively rapid and inexpensive manner in SLE.

Objective: To assess the validity, reliability, and feasibility of the Systemic Lupus Activity Measure-Revised (SLAM-R), the Mexican version of the Systemic Lupus Erythematosus Disease Activity Index (MEX-SLEDAI), and a Modified SLEDAI-2000 (SLEDAI-2K) compared with the SLEDAI-2K in a multiethnic population of patients with SLE., Methods: We studied 92 SLE patients from 3 US geographic areas (Alabama, Texas, and Puerto Rico). Assessment occurred during regular outpatient, inpatient, or study encounters. A trained physician scored the 4 instruments and also assessed disease activity globally [physician global assessment (PGA)]. Convergent (with SLEDAI-2K) and construct validity (with PGA) were determined by Spearman rank (rs) correlation test. Level of agreement between the instruments was assessed using Bland-Altman plots. Discriminant validity (distinguishing clearly active vs mildly/nonactive disease) was assessed considering the SLEDAI-2K (and the PGA) as the gold standard. Feasibility was explored by cost analyses., Results: The SLAM-R, the MEX-SLEDAI, and the Modified SLEDAI-2K were highly correlated with the SLEDAI-2K (rs = 0.566, 0.755, 0.924, respectively) and with the PGA (rs = 0.650, 0.540, 0.634, respectively). The 3 instruments showed good agreement with the SLEDAI-2K (Bland-Altman plots). The Modified SLEDAI-2K had better discriminant validity than the SLAM-R and the MEX-SLEDAI. The Modified SLEDAI-2K was the least expensive instrument., Conclusion: The SLAM-R, the MEX-SLEDAI, and the Modified SLEDAI-2K are adequate options for assessment of SLE disease activity; they are also less costly than the SLEDAI-2K.

Objective: To determine how the American College of Rheumatology (ACR) criteria for the classification of systemic lupus erythematosus (SLE) accrue in a multiethnic cohort of SLE patients., Methods: SLE patients enrolled in a longitudinal study of outcome were analyzed (LUMINA; Lupus in Minorities: Nature versus nurture) for the manner in which ACR criteria manifestations occurred prior to the accrual of 4 of them. Time at which a criterion was said to be present was determined by review of all previously available medical records and interview. Univariable and multivariable Cox proportional hazard models were examined for the association with time to accrual of 4 ACR criteria; results were reported as hazard ratios., Results: There were 103 Texas Hispanic (of Mexican or Central America ancestry) patients, 55 Puerto Rico Hispanics, 176 African Americans, and 137 Caucasians. The mean +/- SD and median (range) time to accrual of 4 ACR criteria were 29.4 +/- 52.0 months and 9.1 (0-328.7) months; time was shortest for the Texas Hispanics (18.4 +/- 42.8 and 5.0 [0-248] months) and longest for the Caucasians (39.9 +/- 59.3 months and 17.7 [0-324.6] months). Arthritis was the most frequent first criterion (34.5%); it was followed by photosensitivity (18.8%). When 2 criteria occurred from the outset, the most frequent combination was arthritis and antinuclear antibody positivity followed by malar rash and photosensitivity. A Cox-regression multivariable model identified Hispanic ethnicity (from Texas) and HLA-DRB1*0301 as predictors of short time to criteria accrual, whereas older age and married/living together were associated with long time to criteria accrual., Conclusion: Significant variability in the evolution of ACR criteria manifestations does occur. Texas Hispanics are more likely to have a rapid evolution of criteria manifestations, but several years may elapse before ACR criteria are accrued.