Development and initial validation of a self-assessed lupus organ damage instrument.

OBJECTIVE: The Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) is a validated instrument for assessing organ damage in systemic lupus erythematosus (SLE). Trained physicians must complete it, thus limiting utility where this is impossible. METHODS: We developed and pilot tested a self-assessed organ damage instrument, the Lupus Damage Index Questionnaire (LDIQ), in 37 SLE subjects and 7 physicians. After refinement, 569 English-speaking SLE subjects and 14 rheumatologists from 11 international SLE clinics participated in validation. Subjects and physicians completed the instruments separately. We calculated sensitivity, specificity, Spearman's correlations, and agreement using the SDI as the gold standard. Six hundred five SLE participants in the community-based National Data Bank for Rheumatic Diseases (NDB) study completed the LDIQ and we assessed correlations with outcome and disability measures. RESULTS: The mean LDIQ score was 3.3 (range 0-16) and the mean SDI score was 1.5 (range 0-9). The LDIQ had a moderately high correlation with the SDI (Spearman's r = 0.50, P < 0.001). Specificities of individual LDIQ items were >80%, except for neuropathy. Sensitivities were variable and lowest for damage, with <1% prevalence. Agreement between the SDI and LDIQ was >85% for all but neuropathy, reduced renal function, deforming arthritis, and alopecia. In the NDB, the LDIQ correlated well with the comorbidity index (r = 0.45), the Short Form 36 physical component scale (r = 0.43), the Medical Research Council dyspnea scale (r = 0.40), disability (r = 0.37), and the Systemic Lupus Erythematosus Activity Questionnaire score (r = 0.37). CONCLUSION: The metric properties of the LDIQ are good compared with the SDI. It has construct validity and correlations with health assessments similar to the SDI. The LDIQ should allow expansion of SLE research. Its ultimate value will be determined in longitudinal studies.

Using the Short Form 6D, as an overall measure of health, to predict damage accrual and mortality in patients with systemic lupus erythematosus: XLVII, results from a multiethnic US cohort.

OBJECTIVE: To determine if overall health status as assessed by the Short Form 6D (SF-6D) index, a preference-based generic measure of health, is associated with the occurrence of damage accrual and mortality in patients with systemic lupus erythematosus (SLE). METHODS: We studied SLE patients (American College of Rheumatology criteria) from the LUpus in MInorities, NAture versus nurture cohort (LUMINA), a longitudinal multiethnic cohort. The contribution of the SF-6D as assessed at enrollment to damage accrual at the last visit and mortality was examined. All variables previously shown to be determinants of damage accrual and mortality and corroborated by univariable analyses were adjusted for in multivariable models (Poisson and Cox proportional hazards regressions, respectively). Damage accrual and mortality were the dependent variables. Similar analyses were performed examining the associations of the Short Form 36 summary measures (physical component summary [PCS], mental component summary [MCS]) with these outcomes. RESULTS: In 552 patients, the SF-6D was negatively associated with damage accrual and mortality in the univariable analyses; the association with damage was confirmed in the multivariable analyses (chi(2) = 9.020, P = 0.002) but the association with mortality was not confirmed (hazard ratio 0.495, 95% confidence interval 0.041-6.038). When the PCS and MCS were evaluated, the PCS, but not the MCS, was found to be associated with damage but not with mortality. CONCLUSION: The SF-6D (and the PCS) as measured early in the disease course were found to independently predict damage accrual at the last visit, but not mortality. Although the SF-6D was originally conceived as a utility measure, it may be used to accurately assess overall health status in patients with SLE.

Systemic lupus erythematosus in a multiethnic US cohort (LUMINA): XXI. Disease activity, damage accrual, and vascular events in pre- and postmenopausal women.

OBJECTIVE: To determine the differences in clinical manifestations, disease activity, damage accrual, and medication use in systemic lupus erythematosus (SLE) patients as a function of menopausal status at disease onset. METHODS: Women with SLE as per the criteria of the American College of Rheumatology, with disease duration of 6 months, and/or oophorectomy, and/or increased follicle-stimulating hormone values for reproductive-age women, and/or treatment with hormone replacement therapy. Patients were divided into premenopausal and postmenopausal categories. Socioeconomic status, cumulative clinical manifestations, disease activity (at study entry or time 0, last visit, and over time), as measured by the Systemic Lupus Activity Measure, and damage accrual, as measured by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index (at time 0 and at last visit) were compared between the 2 groups of women. Multivariable models were then examined making adjustments for all possible known confounders. Dependent variables in the models were renal involvement, damage accrual, arterial vascular events, and venous thrombosis. RESULTS: Five hundred eighteen women from the LUMINA cohort were included; 436 (84.2%) were premenopausal and 82 (15.8%) were postmenopausal. Disease onset after menopause was more common among Caucasians. Renal involvement was more common in premenopausal women, whereas vascular arterial events were more frequent in postmenopausal women. All other disease manifestations, as well as disease activity, were comparable between both groups. The presence of damage accrual at time 0 and study end was more frequent in postmenopausal women. Age, rather than menopausal status, independently contributed to damage accrual, renal involvement, and vascular arterial events in these women. CONCLUSION: A hypoestrogenemic state secondary to menopause appears not to be protective against disease activity and damage accrual. Age rather than menopausal status is a strong independent predictor of damage accrual and of vascular events in women with lupus.

The Systemic Lupus Activity Measure-revised, the Mexican Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), and a modified SLEDAI-2K are adequate instruments to measure disease activity in systemic lupus erythematosus.

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.

Systemic lupus erythematosus in three ethnic groups. XVIII. Factors predictive of poor compliance with study visits.

OBJECTIVE: To determine the baseline factors predictive of poor compliance with study visits in a longitudinal multiethnic lupus cohort study. METHODS: Patients with systemic lupus erythematosus (n = 344) representing a total of 2,069 potential study visits were studied. Of the participants, 24.4% were Hispanic, 43.3% African American, and 32.3% Caucasian. Noncompliance was defined as missing 2 or more study visits. For the purpose of these analyses, visits completed only by review of medical records were considered missing. Baseline socioeconomic-demographic, clinical, and psychosocial features between compliant and noncompliant patients were compared. Variables with P < 0.10 were then entered into a multivariable logistic regression analysis with compliance being the dependent variable. RESULTS: There were 178 compliant and 166 noncompliant patients. Noncompliant patients were more likely to be young, unmarried, of African American ethnicity, live closer to the medical centers, and have longer disease duration and greater disease activity as assessed by the physician than the compliant patients. In the multivariable model, longer disease duration (P = 0.010), higher level of disease activity (P = 0.009), and shorter distance to travel to study visits (P = 0.046) were predictors of noncompliance; their odds ratios and confidence intervals were below 1. CONCLUSIONS: We have identified baseline patient characteristics that may predict noncompliance with study visits (disease duration, disease activity, and distance to the medical center). This information will serve as the basis for developing interventions to curtail noncompliance. Our data may have applicability in other lupus cohort studies.

Systemic lupus erythematosus in three ethnic groups. XIV. Poverty, wealth, and their influence on disease activity.

OBJECTIVE: To determine the impact of wealth on disease activity in the multiethnic (Hispanic, African American, and Caucasian) LUMINA (Lupus in Minorities, Nature versus nurture) cohort of patients with systemic lupus erythematosus (SLE) and disease duration < or =5 years at enrollment. METHODS: Variables (socioeconomic, demographic, clinical, immunologic, immunogenetic, behavioral, and psychological) were measured at enrollment and annually thereafter. Four questions from the Women's Health Initiative study were used to measure wealth. Disease activity was measured with the Systemic Lupus Activity Measure (SLAM). The relationship between the different variables and wealth was then examined. Next, the impact of wealth on disease activity was examined in regression models where the dependent variables were the SLAM score and SLAM global (physician). Variables previously found to impact disease activity plus the wealth questions were included in the models. RESULTS: Questions on income, assets, and debt were found to distinguish patients into groups, wealthier and less wealthy. Less wealthy patients tended to be younger, women, noncaucasian, less educated, unmarried, less likely to have health insurance, and more likely to live below the poverty line. They also tended to have more active disease, more abnormal illness-related behaviors, less social support, and lower levels of self reported mental functioning. None of the wealth questions was retained in the regression models, although other socioeconomic features (such as African American ethnicity, poverty, and younger age) did. CONCLUSIONS: Wealth, per se, does not appear to have an additional predictive value, over and above traditional measures of socioeconomic status, in SLE disease activity.

Systemic lupus erythematosus in three ethnic groups. XI. Sources of discrepancy in perception of disease activity: a comparison of physician and patient visual analog scale scores.

OBJECTIVE: To compare patient's and physician's assessment of disease activity in a multiethnic (Hispanic, African American, and Caucasian) cohort of systemic lupus erythematosus (SLE) patients. METHODS: Three hundred patients with SLE from the LUMINA (Lupus in Minority populations: Nature versus nurture) cohort were included. Disease activity was assessed with the Systemic Lupus Activity Measure (SLAM); patients and physicians assessed disease activity using a 10-cm anchored visual analog scale (VAS). The difference between VAS scores was termed discrepancy (>1 cm was considered a priori clinically relevant). Selected sociodemographic, clinical, behavioral, and psychological variables were examined in relation to discrepancy in univariable and multivariable models adjusting for the physician global VAS score in order to eliminate ceiling and floor effects. RESULTS: A discrepancy was exhibited by 58% of the patients. Abnormal laboratory findings were negatively associated with discrepancy, and poor self-perceived functioning and joint involvement were positively associated with discrepancy. Ethnicity did not account for discrepant perception of disease activity. CONCLUSION: Patients and physicians rate disease activity in SLE differently. Physicians appear to place more emphasis on laboratory features while patients place more emphasis on function.

Systemic lupus erythematosus in three ethnic groups. X. Measuring cognitive impairment with the cognitive symptoms inventory.

OBJECTIVE: To determine the factor structure of the Cognitive Symptoms Inventory (CSI) in patients with systemic lupus erythematosus (SLE) participating in a multiethnic longitudinal study of outcome, the Lupus in Minority populations, Nature versus nurture (LUMINA) study. METHODS: LUMINA patients of Hispanic (n = 48), African American (n = 64), and Caucasian (n = 44) ethnicity who had a study visit (enrollment or followup) between January 1 and September 30, 2000 were included. Patients completed the CSI, a 21-item self-report measure of cognitive function. Sociodemographic, clinical, immunologic, psychosocial, and behavioral variables were ascertained per protocol and as previously described. Data were analyzed with SPSS. The factor structure of the CSI was determined using the principal axis method with oblique rotation as decided by Gorsuch. All factors having an Eigenvalue greater than 1 were considered. A 4-factor solution was derived that accounted for 42.6% of the common variance. The correlations between patient factor scale scores and variables from the demographic, clinical, psychosocial, and behavioral domains were then examined. RESULTS: The four factors and their respective variance are, Attention/Concentration (28.8%), Pattern Recognition/Activity Management (5.7%), Intermediate Memory (4.7%), and Initiation of Executive Functions (3.4%); each factor correlated with the total CSI score. Overall, patients' factor scale scores were positively and significantly correlated with other measures of cognitive dysfunction such as the Systemic Lupus Activity Measure (neuromotor domain) or the Systemic Lupus International Collaborating Clinics Damage Index (neurocognitive impairment), as well as with measures of fatigue, maladaptive coping skills, poor mental functioning, poor social support, and helplessness. They were, however, not correlated with sociodemographic or clinical variables. CONCLUSIONS: In addition to demonstrating that the CSI can be used to measure cognitive impairment in patients with SLE in the research setting, we have determined a 4-factor solution for the CSI that appears to have adequate metric properties. At present, the CSI may best be used as a screen for difficulties in daily activities involving intermediate memory, concentration, attention, and executive function. Nevertheless, further work with the CSI items and factor scales is necessary to establish internal and test-retest reliability of the factor scales; and provide additional evidence of the convergent and predictive validity of these scales in larger samples of patients from each ethnic subgroup.