Evaluation of the EULAR/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus in a Population-Based Registry.

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.

Population-based prevalence and incidence estimates of primary discoid lupus erythematosus from the Manhattan Lupus Surveillance Program.

OBJECTIVE: Epidemiological data for primary discoid lupus erythematosus (pDLE) remain limited, particularly for racial/ethnic populations in the USA. The Manhattan Lupus Surveillance Program (MLSP) is a population-based retrospective registry of cases with SLE and related diseases including pDLE in Manhattan and was used to provide estimates of the prevalence and incidence of pDLE across major racial/ethnic populations. METHODS: MLSP cases were identified from rheumatologists, hospitals and population databases. Two case definitions were used for pDLE: the primary case definition which was any physician diagnosis found in the chart and a secondary case definition which was limited to cases diagnosed by a rheumatologist and/or dermatologist. Rates among Manhattan residents were age-adjusted, and capture-recapture analyses were conducted to assess case under-ascertainment. RESULTS: Based on the primary definition, age-adjusted overall prevalence and incidence rates of pDLE among Manhattan residents were 6.5 and 0.8 per 100 000 person-years, which increased to 9.0 and 1.3 after capture-recapture adjustment. Prevalence and incidence rates were approximately two and six times higher, respectively, among women compared with men (p<0.0001). Higher prevalence was also found among non-Latino blacks (23.5) and Latinos (8.2) compared with non-Latino whites (1.8) and non-Latino Asians (0.6) (p<0.0001). Incidence was highest among non-Latino blacks (2.4) compared with all other racial/ethnic groups. Similar relationships were observed for the secondary case definition. CONCLUSION: Data from the MLSP provide epidemiological estimates for pDLE among the major racial/ethnic populations in the USA and reveal disparities in pDLE prevalence and incidence by sex and race/ethnicity among Manhattan residents.

The Incidence and Prevalence of Adult Primary Sjögren's Syndrome in New York County.

OBJECTIVE: Extant epidemiologic data of primary Sjögren's syndrome (SS) remains limited, particularly for racial/ethnic populations in the US. The Manhattan Lupus Surveillance Program (MLSP) is a population-based retrospective registry of cases of systemic lupus erythematosus and related diseases, including primary SS in Manhattan, New York. The MLSP was used to provide estimates of the incidence and prevalence of primary SS across major racial/ethnic populations. METHODS: MLSP cases were identified from hospitals, rheumatologists, and population databases. Three case definitions were used for primary SS, including physician diagnosis, rheumatologist diagnosis, and modified primary SS criteria. Rates among Manhattan residents were age-adjusted, and capture-recapture analyses were conducted to assess underascertainment of cases. RESULTS: By physician diagnosis, age-adjusted overall incidence and prevalence rates of primary SS among adult Manhattan residents were 3.5 and 13.1 per 100,000 person-years, respectively. Capture-recapture adjustment increased incidence and prevalence rates (4.1 and 14.2 per 100,000 person-years, respectively). Based on physician diagnosis, incidence and prevalence rates were approximately 6 times higher among women than men (P < 0.001). Incidence of primary SS was statistically higher among non-Latina Asian women (10.5) and non-Latina white women (6.2) compared with Latina women (3.2). Incidence was also higher among non-Latina Asian women compared with non-Latina black women (3.3). Prevalence of primary SS did not differ by race/ethnicity. Similar trends were observed when more restrictive case definitions were applied. CONCLUSION: Data from the MLSP revealed disparities among Manhattan residents in primary SS incidence and prevalence by sex and differences in primary SS incidence by race/ethnicity among women. These data also provided epidemiologic estimates for the major racial/ethnic populations in the US.

The Incidence and Prevalence of Systemic Lupus Erythematosus in New York County (Manhattan), New York: The Manhattan Lupus Surveillance Program.

OBJECTIVE: The Manhattan Lupus Surveillance Program (MLSP) is a population-based registry designed to determine the prevalence of systemic lupus erythematosus (SLE) in 2007 and the incidence from 2007 to 2009 among residents of New York County (Manhattan), New York, and to characterize cases by race/ethnicity, including Asians and Hispanics, for whom data are lacking. METHODS: We identified possible SLE cases from hospital records, rheumatologist records, and administrative databases. Cases were defined according to the American College of Rheumatology (ACR) classification criteria, the Systemic Lupus International Collaborating Clinics (SLICC) classification criteria, or the treating rheumatologist's diagnosis. Rates among Manhattan residents were age-standardized, and capture-recapture analyses were conducted to assess case underascertainment. RESULTS: By the ACR definition, the age-standardized prevalence and incidence rates of SLE were 62.2 and 4.6 per 100,000 person-years, respectively. Rates were ∼9 times higher in women than in men for prevalence (107.4 versus 12.5) and incidence (7.9 versus 1.0). Compared with non-Hispanic white women (64.3), prevalence was higher among non-Hispanic black (210.9), Hispanic (138.3), and non-Hispanic Asian (91.2) women. Incidence rates were higher among non-Hispanic black women (15.7) compared with non-Hispanic Asian (6.6), Hispanic (6.5), and non-Hispanic white (6.5) women. Capture-recapture adjustment increased the prevalence and incidence rates (75.9 and 6.0, respectively). Alternate SLE definitions without capture-recapture adjustment revealed higher age-standardized prevalence and incidence rates (73.8 and 6.2, respectively, by the SLICC definition and 72.6 and 5.0 by the rheumatologist definition) than the ACR definition, with similar patterns by sex and race/ethnicity. CONCLUSION: The MLSP confirms findings from other registries on disparities by sex and race/ethnicity, provides new estimates among Asians and Hispanics, and provides estimates using the SLICC criteria.

Assessment of fluorinated steroids to avert progression and mortality in anti-SSA/Ro-associated cardiac injury limited to the fetal conduction system.

OBJECTIVES: Extension of disease beyond the atrioventricular (AV) node is associated with increased mortality in cardiac neonatal lupus (NL). Treatment of isolated heart block with fluorinated steroids to prevent disease progression has been considered but published data are limited and discordant regarding efficacy. This study evaluated whether fluorinated steroids given to manage isolated advanced block prevented development of disease beyond the AV node and conferred a survival benefit. METHODS: In this retrospective study of cases enrolled in the Research Registry for NL, inclusion was restricted to anti-SSA/Ro-exposed cases presenting with isolated advanced heart block in utero who either received fluorinated steroids within 1 week of detection (N=71) or no treatment (N=85). Outcomes evaluated were: development of endocardial fibroelastosis, dilated cardiomyopathy and/or hydrops fetalis; mortality and pacemaker implantation. RESULTS: In Cox proportional hazards regression analyses, fluorinated steroids did not significantly prevent development of disease beyond the AV node (adjusted HR=0.90; 95% CI 0.43 to 1.85; p=0.77), reduce mortality (HR=1.63; 95% CI 0.43 to 6.14; p=0.47) or forestall/prevent pacemaker implantation (HR=0.87; 95% CI 0.57 to 1.33; p=0.53). No risk factors for development of disease beyond the AV node were identified. CONCLUSIONS: These data do not provide evidence to support the use of fluorinated steroids to prevent disease progression or death in cases presenting with isolated heart block.

Maternal and fetal factors associated with mortality and morbidity in a multi-racial/ethnic registry of anti-SSA/Ro-associated cardiac neonatal lupus.

BACKGROUND: Cardiac manifestations of neonatal lupus include conduction disease and, rarely, an isolated cardiomyopathy. This study was initiated to determine the mortality and morbidity of cardiac neonatal lupus and associated risk factors in a multi-racial/ethnic US-based registry to provide insights into the pathogenesis of antibody-mediated injury and data for counseling. METHODS AND RESULTS: Three hundred twenty-five offspring exposed to maternal anti-SSA/Ro antibodies with cardiac neonatal lupus met entry criteria. Maternal, fetal echocardiographic, and neonatal risk factors were assessed for association with mortality. Fifty-seven (17.5%) died, 30% in utero. The probability of in utero death was 6%. The cumulative probability of survival at 10 years for a child born alive was 86%. Fetal echocardiographic risk factors associated with increased mortality in a multivariable analysis of all cases included hydrops and endocardial fibroelastosis. Significant predictors of in utero death were hydrops and earlier diagnosis, and of postnatal death were hydrops, endocardial fibroelastosis, and lower ventricular rate. Isolated heart block was associated with a 7.8% case fatality rate, whereas the concomitant presence of dilated cardiomyopathy or endocardial fibroelastosis quadrupled the case fatality rate. There was a significantly higher case fatality rate in minorities compared with whites, who were at a lower risk of hydrops and endocardial fibroelastosis. Pacing was required in 70%; cardiac transplantation was required in 4 children. CONCLUSION: Nearly one fifth of fetuses who develop cardiac neonatal lupus die of complications predicted by echocardiographic abnormalities consistent with antibody-associated disease beyond the atrioventricular node. The disparity in outcomes observed between minorities and whites warrants further investigation.

Unmet needs among people reported with hepatitis C, New York City.

OBJECTIVE: This project sought to describe unmet needs among patients reported with hepatitis C in New York City. DESIGN: From the New York City Health Department's hepatitis C surveillance database, we randomly selected patients whose positive hepatitis C test was in April or May 2005. In 2006, we interviewed patients by telephone and collected information from their clinicians or by medical record review. SETTING: New York City. PARTICIPANTS: We interviewed 180 of the 387 eligible patients and collected information from clinicians for 145 of the 180 patients. MAIN OUTCOME MEASURES: These included whether patients had understood their clinicians' explanation of their hepatitis C diagnosis, if they had been counseled about not drinking alcohol, information about support group attendance, vaccination against hepatitis A and B, health status, treatment, and other factors. RESULTS: Of the 180 patients, 7% stated that they had not understood their clinicians' explanation of their hepatitis C diagnosis, and 26% said that they had not been counseled about avoiding alcohol. Among the 90% of patients who had not attended a hepatitis support group, 31% were interested in attending. Among the 145 patients with information from clinicians, at least 28% were susceptible to hepatitis A and 18% to hepatitis B. CONCLUSIONS: This hepatitis C surveillance project, with information from patients and clinicians, illustrates a valuable use of a chronic hepatitis C surveillance system. The patients described here had several unmet needs, including hepatitis A and B vaccination, basic information about the virus, support groups, and counseling about preventing further liver damage and preventing transmission to others. Relatively simple and affordable health department activities can address these needs, improving quality of life and decreasing the likelihood of liver disease progression.

Importance of travel in domestically acquired typhoid fever infections: opportunities for prevention and early detection.

Approximately 25% of Salmonella typhi infections in the US occur among nontravelers. Two S. typhi infections in a major US metropolitan city acquired domestically in 2005 were epidemiologically linked to a S. typhi-infected Haitian traveler through their congregation meetings. This investigation highlighted the importance of integrating multiple methods of obtaining epidemiologic information, including laboratory evidence and multiple individual and group interviews. Physicians should consider typhoid fever in their differential diagnosis in communities with close ties to endemic areas. Education of communities whose residents travel regularly to typhoid-endemic areas can reduce infection and transmission risk.