Do adult disease severity subclassifications predict use of cyclophosphamide in children with ANCA-associated vasculitis? An analysis of ARChiVe study treatment decisions.

OBJECTIVE: To determine whether adult disease severity subclassification systems for antineutrophil cytoplasmic antibody-associated vasculitis (AAV) are concordant with the decision to treat pediatric patients with cyclophosphamide (CYC). METHODS: We applied the European Vasculitis Study (EUVAS) and Wegener's Granulomatosis Etanercept Trial (WGET) disease severity subclassification systems to pediatric patients with AAV in A Registry for Childhood Vasculitis (ARChiVe). Modifications were made to the EUVAS and WGET systems to enable their application to this cohort of children. Treatment was categorized into 2 groups, "cyclophosphamide" and "no cyclophosphamide." Pearson's chi-square and Kendall's rank correlation coefficient statistical analyses were used to determine the relationship between disease severity subgroup and treatment at the time of diagnosis. RESULTS: In total, 125 children with AAV were studied. Severity subgroup was associated with treatment group in both the EUVAS (chi-square 45.14, p < 0.001, Kendall's tau-b 0.601, p < 0.001) and WGET (chi-square 59.33, p < 0.001, Kendall's tau-b 0.689, p < 0.001) systems; however, 7 children classified by both systems as having less severe disease received CYC, and 6 children classified as having severe disease by both systems did not receive CYC. CONCLUSION: In this pediatric AAV cohort, the EUVAS and WGET adult severity subclassification systems had strong correlation with physician choice of treatment. However, a proportion of patients received treatment that was not concordant with their assigned severity subclass.

Increased sensitivity of the European medicines agency algorithm for classification of childhood granulomatosis with polyangiitis.

OBJECTIVE: Granulomatosis with polyangiitis (Wegener's; GPA) and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare in childhood and are sometimes difficult to discriminate. We compared use of adult-derived classification schemes for GPA against validated pediatric criteria in the ARChiVe (A Registry for Childhood Vasculitis e-entry) cohort, a Childhood Arthritis and Rheumatology Research Alliance initiative. METHODS: Time-of-diagnosis data for children with physician (MD) diagnosis of AAV and unclassified vasculitis (UCV) from 33 US/Canadian centers were analyzed. The European Medicines Agency (EMA) classification algorithm and European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES) and American College of Rheumatology (ACR) criteria for GPA were applied to all patients. Sensitivity and specificity were calculated (MD-diagnosis as reference). RESULTS: MD-diagnoses for 155 children were 100 GPA, 25 microscopic polyangiitis (MPA), 6 ANCA-positive pauciimmune glomerulonephritis, 3 Churg-Strauss syndrome, and 21 UCV. Of these, 114 had GPA as defined by EMA, 98 by EULAR/PRINTO/PRES, and 87 by ACR. Fourteen patients were identified as GPA by EULAR/PRINTO/PRES but not by ACR; 3 were identified as GPA by ACR but not EULAR/PRINTO/PRES. Using the EMA algorithm, 135 (87%) children were classifiable. The sensitivity of the EMA algorithm, the EULAR/PRINTO/PRES, and ACR criteria for classifying GPA was 90%, 77%, and 69%, respectively, with specificities of 56%, 62%, and 67%. The relatively poor sensitivity of the 2 criteria related to their inability to discriminate patients with MPA. CONCLUSION: EULAR/PRINTO/PRES was more sensitive than ACR criteria in classifying pediatric GPA. Neither classification system has criteria for MPA; therefore usefulness in discriminating patients in ARChiVe was limited. Even when using the most sensitive EMA algorithm, many children remained unclassified.

Assessing the performance of the Birmingham Vasculitis Activity Score at diagnosis for children with antineutrophil cytoplasmic antibody-associated vasculitis in A Registry for Childhood Vasculitis (ARChiVe).

OBJECTIVE: There are no validated tools for measuring disease activity in pediatric vasculitis. The Birmingham Vasculitis Activity Score (BVAS) is a valid disease activity tool in adult vasculitis. Version 3 (BVAS v.3) correlates well with physician's global assessment (PGA), treatment decision, and C-reactive protein in adults. The utility of BVAS v.3 in pediatric vasculitis is not known. We assessed the association of BVAS v.3 scores with PGA, treatment decision, and erythrocyte sedimentation rate (ESR) at diagnosis in pediatric antineutrophil cytoplasmic antibody-associated vasculitis (AAV). METHODS: Children with AAV diagnosed between 2004 and 2010 at all ARChiVe centers were eligible. BVAS v.3 scores were calculated with a standardized online tool (www.vasculitis.org). Spearman's rank correlation coefficient (r(s)) was used to test the strength of association between BVAS v.3 and PGA, treatment decision, and ESR. RESULTS: A total of 152 patients were included. The physician diagnosis of these patients was predominantly granulomatosis with polyangiitis (n = 99). The median BVAS v.3 score was 18.0 (range 0-40). The BVAS v.3 correlations were r(s) = 0.379 (95% CI 0.233 to 0.509) with PGA, r(s) = 0.521 (95% CI 0.393 to 0.629) with treatment decision, and r(s) = 0.403 (95% CI 0.253 to 0.533) with ESR. CONCLUSION: Applied to children with AAV, BVAS v.3 had a weak correlation with PGA and moderate correlation with both ESR and treatment decision. Prospective evaluation of BVAS v.3 and/or pediatric-specific modifications to BVAS v.3 may be required before it can be formalized as a disease activity assessment tool in pediatric AAV.

Anesthesia for intra-articular corticosteroid injections in juvenile idiopathic arthritis: A survey of pediatric rheumatologists.

OBJECTIVE: To determine the methods of anesthesia currently being used by pediatric rheumatologists when performing intra-articular corticosteroid injections (IACI). STUDY DESIGN: A questionnaire was emailed to all members of the Childhood Arthritis & Rheumatology Research Alliance, a pediatric rheumatology research network in North America. The questionnaire consisted of 11 questions ranging from procedure technique, treatments prescribed for topical anesthesia and oral analgesia, and factors that might affect procedural pain. RESULTS: Seventy-four of 161 physicians (46%) responded to the questionnaire. On average, each physician injected 33 children (median 25, range 1-160) and 43 joints (median 30, range 1-150) yearly. Local anesthesia was used in children on average >/= 8 years (range 2-16 years), with general anesthesia being more frequently used for younger children. All respondents used local anesthesia. The most commonly used methods of local anesthesia were EMLA((R) )cream plus subcutaneous lidocaine (58.8%), ethyl chloride spray only (39.7%), EMLA((R) )cream only (33.8%), subcutaneous lidocaine only (25%), and lidocaine iontophoresis only (11.8%). Buffering of the lidocaine was routinely done only 7.4% of the time. CONCLUSION: Although pediatric rheumatologists in North America perform IACI on a large number of patients each year, a wide variety of methods are used to deliver local anesthesia with no accepted standard of care. More studies are needed to determine the optimal method of local anesthesia delivery to minimize pain associated with IACI.

Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood.

OBJECTIVE: To compare the criteria for Wegener's granulomatosis (WG) of the American College of Rheumatology (ACR) with those of the European League Against Rheumatism/Pediatric Rheumatology European Society (EULAR/PRES) in a cohort of children with WG and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs), and to describe the interval to diagnosis, presenting features, and initial treatment for WG. METHODS: Eligible patients had been diagnosed by site rheumatologists (termed the "MD diagnosis") since 2004. This diagnosis was used as a reference standard for sensitivity and specificity testing of the 2 WG classification criteria. Descriptive analyses were confined to ACR-classified WG patients. RESULTS: MD diagnoses of 117 patients (82 of whom were female) were WG (n = 76), microscopic polyangiitis (n = 17), ANCA-positive pauci-immune glomerulonephritis (n = 5), Churg-Strauss syndrome (n = 2), and unclassified vasculitis (n = 17). The sensitivities of the ACR and EULAR/PRES classification criteria for WG among the spectrum of AAVs were 68.4% and 73.6%, respectively, and the specificities were 68.3% and 73.2%, respectively. Two more children were identified as having WG by the EULAR/PRES criteria than by the ACR criteria. For the 65 ACR-classified WG patients, the median age at diagnosis was 14.2 years (range 4-17 years), and the median interval from symptom onset to diagnosis was 2.7 months (range 0-49 months). The most frequent presenting features by organ system were constitutional (89.2%), pulmonary (80.0%), ear, nose, and throat (80.0%), and renal (75.4%). Fifty-four patients (83.1%) commenced treatment with the combination of corticosteroids and cyclophosphamide, with widely varying regimens; the remainder received methotrexate alone (n = 1), corticosteroids alone (n = 4), or a combination (n = 6). CONCLUSION: The EULAR/PRES criteria minimally improved diagnostic sensitivity and specificity for WG among a narrow spectrum of children with AAVs. Diagnostic delays may result from poor characterization of childhood WG. Initial therapy varied considerably among participating centers.

Impact of immigration on the clinical expression of systemic lupus erythematosus: a comparative study of Hispanic patients residing in the USA and Mexico.

OBJECTIVE: To compare the socio-economic characteristics, clinical features and health-related quality of life in Hispanic SLE patients residing in Mexico and in the Southwest USA (Mexican and Texan, herein). METHODS: Mexican and Texan SLE patients (fulfilling ACR criteria) participating in separate longitudinal outcome studies were evaluated. Texan patients were randomly chosen to match total disease duration with the Mexican patients. Cross-sectional data for the Mexican patients were obtained by a US-trained investigator who had previously participated in data collection for the cohort to which the Texan patients belonged. Socio-economic and -demographic characteristics, clinical characteristics, disease activity (with SLAM-Revised), damage accrual (with SLICC/ACR Damage Index) and self-reported function (with Short Form-36) were compared between the two groups. RESULTS: Seventy Mexican patients were matched with either one or two Texan patients (n = 94) for a total of 164 patients. Mexican patients were younger. In age-adjusted analyses, the Mexican patients were more educated, had better health-related quality of life and overall less systemic SLE manifestations. Mexican patients were exposed more frequently to AZA. CONCLUSIONS: Texan patients had more severe disease than the Mexican patients. In multivariable analyses, Texan Hispanic ethnicity was significantly associated with high disease activity, but significance was not reached for damage. The discrepant findings observed between these two Hispanic groups of SLE patients may reflect socio-economic or biological factors. Given the global phenomenon of immigration, rheumatologists should be aware of the overall course and outcome of immigrant SLE patients if undesirable outcomes are to be prevented.

Establishment of a pilot pediatric registry for chronic vasculitis is both essential and feasible: a Childhood Arthritis and Rheumatology Alliance (CARRA) survey.

OBJECTIVE: To identify the need for, and feasibility of, establishing a web-based USA/Canadian registry of children with chronic systemic vasculitis--an otherwise insufficiently studied population. METHODS: Physician members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA; n = 126) were invited to complete 2 surveys exploring vasculitis-related experience, beliefs about childhood versus adult vasculitis, and commitment to contribute patients to a prospective registry. Diagnoses included Wegener's granulomatosis (WG), childhood polyarteritis nodosa, microscopic polyangiitis (MPA), Takayasu's arteritis, primary angiitis of the central nervous system (PACNS), vasculitis, and unclassified vasculitis. RESULTS: One or both surveys were completed by 102 (81%) physicians. Almost half of first-survey respondents had been in practice for > 15 years. Collective estimated lifetime experience was >1500 patients (WG and unclassified vasculitis were the most common diagnoses). Three hundred seventeen children with vasculitis were seen in the year preceding the survey, with most physicians seeing only 2-5 patients. The majority of respondents believed that childhood vasculitis differed from adult disease, particularly with respect to classification criteria and disease activity markers. Fifty-nine members committed to contribute 2 years' data (approximately 120 patients) to a pilot registry limited to time of diagnosis, focusing on WG, MPA, Churg-Strauss syndrome, PACNS, and unclassified vasculitis. CONCLUSION: We obtained overwhelming consensus from an experienced body of pediatric rheumatologists on the need to study childhood-onset vasculitis independently from adult disease, together with commitment from sufficient members to prospectively contribute 2 years' data to a limited pilot registry to answer some basic questions about presenting and diagnostic features and initial treatment practices at disease onset.

A trial of contraceptive methods in women with systemic lupus erythematosus.

BACKGROUND: The effects of estrogen-containing contraceptives on disease activity in women with systemic lupus erythematosus have not been determined. METHODS: We conducted a single-blind clinical trial involving 162 women with systemic lupus erythematosus who were randomly assigned to combined oral contraceptives, a progestin-only pill, or a copper intrauterine device (IUD). Disease activity was assessed at 0, 1, 2, 3, 6, 9, and 12 months according to the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). The primary outcome was global disease activity, which we estimated by measuring the area under the SLEDAI curve. Secondary outcomes included the maximum SLEDAI score, change in SLEDAI score, incidence of lupus flares, median time to first flare, systemic lupus erythematosus treatment, and adverse events. The results were analyzed by the intention-to-treat method. RESULTS: At baseline, all demographic features and disease characteristics were similar in the three groups. The mean (+/-SD) SLEDAI score was 6.1+/-5.6 in the group assigned to combined oral contraceptives, 6.4+/-4.6 in the group assigned to the progestin-only pill, and 5.0+/-5.3 in the group assigned to the IUD (54 patients in each group) (P=0.36). Disease activity remained mild and stable in all groups throughout the trial. There were no significant differences among the groups during the trial in global or maximum disease activity, incidence or probability of flares, or medication use. The median time to the first flare was three months in all groups. Thromboses occurred in four patients (two in each of the two groups receiving hormones), and severe infections were more frequent in the IUD group. One patient receiving combined oral contraceptives died from amoxicillin-related severe neutropenia. CONCLUSIONS: Global disease activity, maximum SLEDAI score, incidence of flares, time to first flare, and incidence of adverse events were similar among women with systemic lupus erythematosus, irrespective of the type of contraceptive they were using.

Systemic lupus erythematosus in a multiethnic US cohort (LUMINA). XXIII. Baseline predictors of vascular events.

OBJECTIVE: To determine the baseline (time 0) risk factors associated with the subsequent occurrence of vascular events in a multiethnic US cohort (LUMINA [LUpus in MInorities: NAture versus nurture]) of patients with systemic lupus erythematosus (SLE). METHODS: Five hundred forty-six LUMINA patients were assessed at time 0 for traditional and nontraditional (disease-related) risk factors for vascular events. These were defined as 1) cardiovascular (myocardial infarction and/or definite or classic angina and/or the undergoing of a vascular procedure for myocardial infarction [coronary artery bypass graft]), 2) cerebrovascular (stroke), and 3) peripheral vascular (arterial claudication and/or gangrene or significant tissue loss and/or arterial thrombosis in peripheral arteries). The observation time (followup time in the cohort) was the interval between time 0 and the last visit. The unit of analysis was the patient and not each vascular event. Variables at time 0 and vascular events were examined by univariable and multivariable (logistic and Cox proportional hazards regression) analyses. Age, sex, ethnicity, followup time, and all known risk factors for the occurrence of vascular events were included in the model. RESULTS: Thirty-four patients (6.2%) developed one or more vascular event after time 0. The overall median duration of followup in the cohort was 73.8 months (range 10.8-111.3 months). Vascular events (13 cardiovascular, 18 cerebrovascular, 5 peripheral vascular) occurred in 7 Hispanics from Texas (6.5%), 1 Hispanic from Puerto Rico (1.2%), 15 African Americans (7.5%), and 11 Caucasians (7.1%). The mean total number of traditional risk factors was significantly higher in patients who developed vascular events than in those who did not (7.1 versus 5.6). Independent predictors of vascular events were older age, current smoking status, longer followup time, elevated serum levels of C-reactive protein (CRP), and the presence of any antiphospholipid antibody. The same variables were identified when time-dependent analyses were performed, although azathioprine use was also found to be a contributing factor. CONCLUSION: Smoking, previously not reported in SLE, emerged as a predictor of vascular events and should be strongly discouraged. Antiphospholipid antibodies and CRP support the role of inflammation and autoimmunity in the development of accelerated atherosclerosis in SLE. Ethnicity was not associated with vascular events in our patients.

Systemic lupus erythematosus in a multiethnic US cohort (LUMINA): XXII. Predictors of time to the occurrence of initial damage.

OBJECTIVE: To examine the factors predisposing to initial damage in patients in the LUMINA (lupus in minorities: nature versus nurture) cohort, a multiethnic cohort of patients with systemic lupus erythematosus (SLE) in the US. METHODS: One hundred fifty-eight LUMINA patients with no damage at baseline (time 0) according to the Systemic Lupus International Collaborating Clinics Damage Index (SDI) and with disease duration > 6 months were followed up for a median of 24 months (range 5-112 months). Damage was assessed from time 0 to the last visit. Predictors of time to initial damage were examined by univariable and multivariable Cox proportional hazard regression models. Results were reported as hazard ratios (HRs); HR values > or = 1 indicated a shorter time to initial damage, and values < 1 indicated a longer time. RESULTS: Initial damage occurred in 54 patients (34%), of whom 21 were Hispanics from Texas (39%), 2 were Hispanics from Puerto Rico (4%), 21 were African Americans (39%), and 10 were Caucasians (19%). The most frequently observed initially involved SDI domains (and items) were as follows: renal (primarily proteinuria) in Hispanics from Texas and African Americans, integument (primarily scarring alopecia) in Hispanics from Puerto Rico, and ocular (primarily cataracts) in Caucasians. By multivariable analyses, independent predictors of a shorter time to initial damage were Hispanic ethnicity from Texas (HR 2.11, 95% confidence interval [95% CI] 1.15-3.88), greater disease activity according to the Systemic Lupus Activity Measure (HR 1.09, 95% CI 1.04-1.15), the occurrence of thrombotic events in visceral and/or peripheral veins or arteries (HR 7.66, 95% CI 2.13-27.51), and prednisone at a dosage of < 10 mg/day (HR 2.53, 95% CI 1.15-5.55). Prednisone at a dosage of 10-30 mg/day was found to be protective against the occurrence of initial damage (HR 0.46, 95% CI 0.22-0.96). CONCLUSION: Given that damage is a predictor of further damage, identifying the factors that may herald the occurrence of initial damage has very practical implications for the management of patients with SLE. These results need to be considered when evaluating therapies for SLE.

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. XIX. Natural history of the accrual of the American College of Rheumatology criteria prior to the occurrence of criteria diagnosis.

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.

What have we learned from a 10-year experience with the LUMINA (Lupus in Minorities; Nature vs. nurture) cohort? Where are we heading?

Recently, there has been an awareness of the variable phenotypic expression of numerous disorders between individuals from different ethnicities, systemic lupus erythematosus (SLE) one of them. These disparities probably arise from the interaction between genetic and non-genetic (environmental, socioeconomic-demographic, cultural and behavioral) factors. To delineate the influence of these factors on SLE outcome, we established a multiethnic (Hispanic, African American and Caucasian) United States (US) early cohort (<5 years disease duration). Ten years later, interesting data have emerged from the LUMINA (Lupus in Minorities: Nature vs. nurture) cohort. For example, African Americans and Hispanics from Texas have a more severe disease than Caucasians and Hispanics from Puerto Rico. Lack of private insurance, acute SLE onset, expression of HLA-DRB1*01 (DR1) and C4A*3 alleles were associated with higher disease activity, whereas age, the number of American College of Rheumatology criteria met, disease activity, corticosteroid use and abnormal illness behaviors were consistent predictors of damage. In turn, damage and poverty were found to predict mortality. We now plan to apply new approaches (genetic admixture) to deconfound the complex interaction between genetic and non-genetic factors influencing SLE outcome. These data may have impact on the development of policies aimed at eliminating health disparities in the US.

Systemic lupus erythematosus in a multiethnic lupus cohort (LUMINA). XVII. Predictors of self-reported health-related quality of life early in the disease course.

OBJECTIVE: To determine the baseline factors predictive of self-reported health-related quality of life (HRQOL) early in the course of systemic lupus erythematosus patients (SLE) from a multiethnic LUMINA (Lupus in Minorities: Nature versus nurture) cohort. METHODS: LUMINA patients with > or =2 visits were studied. Self-reported HRQOL was examined with the 8 subscales and 2 summary measures (the Physical Component Summary [PCS], and the Mental Component Summary [MCS]) of the Short Form 36 (SF-36). Bivariable and multivariable analyses were done with the PCS, MCS and 8 subscales as the dependent variables. The analyses were performed including and excluding the corresponding SF-36 measure from the independent variables. Age, sex, and ethnicity were included in all models. Time was modeled in all regressions. RESULTS: A total of 1,351 visits (346 patients [80 Hispanics-Texas, 34 Hispanics-Puerto Rico, 126 African Americans, and 106 Caucasians]) were included in these analyses. Mean +/- SD PCS and MCS scores were 36.7 +/- 12.0 and 46.6 +/- 11.5, respectively. The scores for the eight subscales of the SF-36 were also lower than those for the general population. Baseline SF-36 measures were highly predictive of subsequent HRQOL. In the same set of regressions, older age was found to consistently predict poor self-reported HRQOL whereas fibromyalgia, helplessness, fatigue, and abnormal illness-related behaviors were also predictive, but less consistently. Estimated adjusted variances in these regressions ranged from 23% (Role-Emotional [RE]) to 43% (Physical Functioning [PF]). CONCLUSION: In patients with SLE, poor baseline HRQOL was highly predictive of subsequent poor HRQOL. Other predictive variables of poor functioning were primarily psychological/behavioral and socioeconomic-demographic.

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.

Ethnic disparities in patients with systemic lupus erythematosus.

Ethnic disparities in health care have been historically well documented, but their causes still remain poorly explained. In the US, ethnic minorities have a higher incidence and prevalence of systemic lupus erythematosus and also experience less favorable outcomes when compared with the Caucasian majority. These discrepancies can be explained, at least in part, by genetic-related ethnic factors; however, nongenetic factors emerging from differences in socioeconomic status and related individual social (poverty, limited access to quality health care) and cultural characteristics (inadequate health belief patterns, distrust in medical institutions) are also likely to contribute to these discrepancies. A comprehensive recognition of current unfavorable, but modifiable, circumstances will provide the framework to develop strategic approaches toward eliminating existent disparities in health, including those occurring in patients with systemic lupus erythematosus.