Cristina Drenkard

Vasculitis in systemic lupus erythematosus

We studied the frequency, location, clinical and histopathological features, associated manifestations, and prognosis of vasculitides in a cohort of 667 SLE patients. Exclusion of patients with previous vasculitis or insufficient information left 540 patients, 194 of whom had vasculitis (incidence density: 0.053 new cases/person/year, cumulative incidence of 0.051 at one year, 0.232 at 5 years and 0.411 at 10 years). Vasculitis was confirmed by biopsy in 46 cases, by arteriography in five, and by both in three. A single episode of vasculitis occurred in 119 and two or more in 75 patients. Vasculitis was cutaneous in 160, visceral in 24, both in 10. In the first episode of cutaneous vasculitides, 111 had punctuate lesions, 32 palpable purpura, 6 urticaria, 6 ulcers, 8 papules, 5 erythematous plaques or macules confirmed with biopsy, 2 erythema with necrosis, and 1 panniculitis (plus small vessel vasculitis). Of 29 with visceral vasculitis in the first episode, 19 had mononeuritis multiplex, 5 digital necrosis, 3 large artery vasculitis of limbs, one mesenteric, and one coronary. More than one type could appear simultaneously or in subsequent episodes. Patients with vasculitis had longer disease duration and followup, younger age of onset of SLE, and were more frequently males than those without. Lupus manifestations associated with vasculitis in univariate logistic regression included myocarditis, psychosis, Raynauds phenomenon, serositis, leukopenia, lymphopenia and pleuritis. Vasculitis also associated with the antiphospholipid syndrome. The strength of this association increased when patients with vasculitis confirmed by biopsy and/or arteriography were considered separately. Visceral vasculitis associated with increased mortality when controlled for age of onset and nephropathy.

The Incidence and Prevalence of Systemic Lupus Erythematosus, 2002–2004: The Georgia Lupus Registry

The Georgia Lupus Registry is a population‐based registry designed to improve our ability to estimate the incidence and prevalence of systemic lupus erythematosus (SLE) in a large population.

Systemic lupus erythematosus in males. A study of 107 Latin American patients.

Clinical and laboratory features were analyzed in 107 Latin American male patients with systemic lupus erythematosus (SLE) who were compared with a group of 1,209 Latin American female patients with SLE to determine the presence of gender-associated differences. Males had an increased prevalence of renal disease, vascular thrombosis, and the presence of anti-dsDNA antibodies, as well as the use of moderate to high doses of corticosteroids, compared with female SLE patients. Although there was no difference in mortality from all causes, SLE-related mortality was higher in the male group. All these findings are consistent with a more severe disease in Latin American males than in female patients from the same region.

Serum anti-β2-glycoprotein-I and anticardiolipin antibodies during thrombosis in systemic lupus erythematosus patients

Abstract PURPOSE: Antibodies to β2-glycoprotein-I are more strongly associated with clinical antiphospholipid syndrome than are anticardiolipin antibodies. We previously found a decrease in anticardiolipin antibodies at the time of thrombosis in 6 patients with systemic lupus erythematosus (SLE). We therefore sought to determine the prevalence and levels of antibodies to β2-glycoprotein-I and to cardiolipin before, during, and after thrombosis in patients with SLE, and to compare them with patients who did not have thrombosis. METHODS: We studied 24 patients with SLE who had at least one episode of thrombosis and 102 patients with SLE without thrombosis. Serum anticardiolipin antibodies were measured by conventional enzyme-linked immunosorbent assay (ELISA) using newborn calf serum as the blocking agent. Serum anti-β2-glycoprotein-I antibodies were measured by ELISA on nonirradiated plates, using purified human β2-glycoprotein-I without phospholipid. RESULTS: All patients with thrombosis had anti-β2-glycoprotein-I antibodies, compared with only 17% of controls (P CONCLUSION: Anti-β2-glycoprotein-I antibodies are strongly associated with thrombosis in patients with SLE. The decrease of anti-β2-glycoprotein-I levels at the time of thrombosis may indicate a pathogenic role. This antibody may also be a marker of predisposition for thrombosis in these patients.

REMISSION OF SYSTEMIC LUPUS ERYTHEMATOSUS

: The occurrence and characteristics of remissions in patients with systematic lupus erythematosus (SLE) have not been determined. We therefore studied this in a cohort of 667 patients and found that 156 patients had achieved at least 1 period of 1 year or more of treatment-free clinical remission. This represents an incidence density of 0.028 new cases/person/year. Remission occurred within the first 2 years of disease in 62 patients. The mean duration of first remission was 4.6 years (range, 1-21 yr), and 81 patients were still in the initial remission up until cutoff time. Half of the remaining 75 patients who flared after achieving remission have not entered again in remission. Twenty-six of the 38 patients who did remained in remission, and the remaining 12 had subsequent flares and remissions. Treatment-free remission accounted for a mean of 5.8 years, corresponding to half the time of follow-up. Remission was not limited to patients with mild disease: at least 41 patients achieved remission despite renal involvement, 19 had had neuropsychiatric lupus, 15 had had thrombocytopenia, and 8 had had hemolytic anemia. We also found that the longer the time lapse between the initial manifestation and the diagnosis of SLE, the less likely it was for a patient to enter into remission. There was a continuous increase in likelihood of achieving a first remission from the beginning of disease up to 30 years of disease duration, when it reached 70%. Patients who achieved remission had increased survival, independently of the effect of other disease manifestations that cause increased mortality. We conclude that a significant proportion of patients with SLE, including those with severe organ involvement, may become symptom-free and in need of no more medication, perhaps indefinitely. Our findings support the notion that, in general, SLE is a more benign disease than previously considered.

Population-based lupus registries: Advancing our epidemiologic understanding

Introduction Without a new medication approved for systemic lupus erythematosus (SLE) by the Food and Drug Administration in more than 40 years, there has been a recent flurry of research activity and clinical trials. However, a basic epidemiologic understanding of SLE, which is necessary to understand the full clinical spectrum and population burden, lags behind. Estimates of the incidence and prevalence of SLE in the US have varied widely and are outdated (Table 1). This is likely due to the use of different case definitions, limited sources for case ascertainment, small source populations, and different demographic groups targeted, as well as the protean characteristics of the disease, poor reliability of self-report, lack of reliability in diagnosis and coding in health system databases, and issues related to access to health care by high-risk populations. Estimates for other types of lupus (e.g., primary discoid lupus) are even less well defined. Two ongoing population-based lupus registries are currently addressing many of these issues, using methods that take advantage of novel federal, state, and local partnerships. In keeping with the goals of the “National Arthritis Action Plan: A Public Health Strategy” (1), the Centers for Disease Control and Prevention (CDC) Arthritis Program in 2002 competitively funded small grants in the health departments of 3 states to plan a population-based registry to better define the incidence and prevalence of diagnosed lupus and to better characterize individuals with this disease. Areas with a population of more than 1 million and with a relatively large African American proportion were eligible. In 2003, state health departments in Georgia and Michigan along with their academic partners, Emory University and the University of Michigan, were competitively awarded funding to perform this research. This article provides an overview of the methods used in these registries, focusing primarily on SLE and emphasizing aspects unique in the field of lupus epidemiology. We also report briefly on our progress and discuss future directions.

The number of flares patients experience impacts on damage accrual in systemic lupus erythematosus: data from a multiethnic Latin American cohort

Purpose To determine the association between the number of flares systemic lupus erythematosus (SLE) patients experience and damage accrual, independently of other known risk factors. Methods SLE patients (34 centres, nine Latin American countries) with a recent diagnosis (≤2 years) and ≥3 evaluations were studied. Disease activity was ascertained with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and damage with the SLICC/ACR Damage Index (SDI). Flare was defined as an increase ≥4 points in the SLEDAI between two study visits. An ambidirectional case- crossover design was used to determine the association between the number of flares and damage accrual. Results 901 patients were eligible for the study; 500 of them (55.5%) experienced at least one flare, being the mean number of flares 0.9 (SD: 1.0). 574 intervals from 251 patients were included in the case-crossover design since they have case and control intervals, whereas, the remaining patients did not. Their mean age at diagnosis was 27.9 years (SD: 11.1), 213 (84.9%) were women. The mean baseline SDI and SLEDAI were 1.3 (1.3) and 13.6 (8.1), respectively. Other features were comparable to those of the entire sample. After adjusting for possible confounding variables, the number of flares, regardless of their severity, was associated with damage accrual (SDI) OR 2.05, 95% CI 1.43 to 2.94, p<0.001 (OR 2.62, 95% CI 1.31 to 5.24, p=0.006 for severe and OR 1.91, 95% CI 1.28 to 2.83, p=0.001for mild-moderate). Conclusions The number of flares patients experience, regardless of their severity, increases the risk of damage accrual, independently of other known risk factors.

Benefits of a self-management program in low-income African-American women with systemic lupus erythematosus: results of a pilot test

Minorities with systemic lupus erythematosus (SLE) are at high risk of poor disease outcomes and may face challenges in effectively self-managing multiple health problems. The Chronic Disease Self-Management Program (CDSMP) is an evidence-based intervention that improves the health of people with chronic illnesses. Although the CDSMP is offered by organizations throughout the United States and many countries around the world, it has not been tested among SLE patients. We pilot tested the benefits of the CDSMP in low-income African American patients with SLE. CDSMP workshops were delivered to 49 African American women with SLE who received medical care at a public lupus clinic in Atlanta, Georgia, US. We compared pre-post CDSMP changes (from baseline to 4 months after the start of the intervention) in health status, self-efficacy and self-management behaviors using self-reported measures. Additionally, we assessed health care utilization changes using electronic administrative records in the 6-month periods before and after the intervention. We observed significant improvements post-intervention in the SF-36 physical health component summary (mean change = 2.4, p = 0.032); self-efficacy (mean change = 0.5, p = 0.035); and several self-management behaviors: cognitive symptoms management (mean change = 0.3, p = 0.036); communication with physicians (mean change = 0.4, p = 0.01); and treatment adherence (mean change = 0.4, p = 0.01). The median number of outpatient visits decreased from 3 to 1 (p < .0001). The CDSMP is a promising intervention for low-income African Americans with SLE. It is an inexpensive program with growing availability around the world that should be further evaluated as a resource to improve patient-centered outcomes and decrease health service utilization among SLE patients.

Validity of a self-administered version of the brief index of lupus damage in a predominantly African American systemic lupus erythematosus cohort.

To assess the reliability and criterion and construct validity of the self‐administered Brief Index of Lupus Damage (SA‐BILD), a patient‐reported measure of organ damage in systemic lupus erythematosus (SLE).

Primary preventive services in patients with systemic lupus erythematosus: Study from a population-based sample in Southeast U.S. ☆

OBJECTIVES Systemic lupus erythematosus (SLE) patients are at risk for complications that can be mitigated by appropriate preventive care. We examined the receipt of immunizations, cancer screening, and cardiovascular risk preventive services in a predominantly Black cohort of SLE patients from the Southeast U.S. To identify gaps in primary preventive services (PPS) that might be specific to SLE as opposed to local health system factors, we used as reference a population-based sample from the same area. METHODS A cross-sectional design was used to characterize the percentage of PPS received by 751 SLE patients from Atlanta, GA, and 9040 subjects from the same community, of whom 938 had diabetes. Factors associated with the receipt of PPS were examined with multivariable analysis of variance. RESULTS Approximately 65% of recommended PPS were provided to the SLE, overall community (OC), and diabetes samples. However, only 22.5%, 45.7%, and 27.6% of SLE, OC, and diabetes subjects, respectively, received all recommended services. Factors associated with a higher percentage of PPS received by SLE patients included older age (63.6% if age ≥65 years, 45.8% if age between 18 and 35 years), having medical insurance (61.1% for insured, 49.7% for uninsured), having a primary care physician (PCP) (59.0% if patient had PCP, 51.8% if patient did not have PCP), and being a non-smoker (61.9% for non-smokers, 49.9% for smokers). CONCLUSIONS Less than one-quarter of SLE patients from a southeast U.S. community received all the recommended services that were studied. Further research is warranted to unravel the barriers that prevent SLE patients from reaching appropriate standards of preventive care.