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13-year-old Girl with Fevers

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      Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus.

      The Systemic Lupus International Collaborating Clinics (SLICC) group revised and validated the American College of Rheumatology (ACR) systemic lupus erythematosus (SLE) classification criteria in order to improve clinical relevance, meet stringent methodology requirements, and incorporate new knowledge regarding the immunology of SLE. The classification criteria were derived from a set of 702 expert-rated patient scenarios. Recursive partitioning was used to derive an initial rule that was simplified and refined based on SLICC physician consensus. The SLICC group validated the classification criteria in a new validation sample of 690 new expert-rated patient scenarios. Seventeen criteria were identified. In the derivation set, the SLICC classification criteria resulted in fewer misclassifications compared with the current ACR classification criteria (49 versus 70; P = 0.0082) and had greater sensitivity (94% versus 86%; P < 0.0001) and equal specificity (92% versus 93%; P = 0.39). In the validation set, the SLICC classification criteria resulted in fewer misclassifications compared with the current ACR classification criteria (62 versus 74; P = 0.24) and had greater sensitivity (97% versus 83%; P < 0.0001) but lower specificity (84% versus 96%; P < 0.0001). The new SLICC classification criteria performed well in a large set of patient scenarios rated by experts. According to the SLICC rule for the classification of SLE, the patient must satisfy at least 4 criteria, including at least one clinical criterion and one immunologic criterion OR the patient must have biopsy-proven lupus nephritis in the presence of antinuclear antibodies or anti-double-stranded DNA antibodies. Copyright © 2012 by the American College of Rheumatology.
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        Epidemiology of systemic lupus erythematosus: a comparison of worldwide disease burden.

        Systemic lupus erythematosus (SLE) is a disease of multifactorial etiology. Quantifying the burden of SLE across different countries can clarify the role of genetic, environmental and other causative factors in the natural history of the disease, and to understand its clinical and societal consequences. The aim of this study is to summarize data on SLE incidence and prevalence in the USA, Europe, Asia, and Australia. An extensive review of electronic resources (PubMed and MedLine) and medical journals was conducted to identify published studies on SLE incidence and prevalence over the period of 1950-early 2006. Researchers in the countries of interest provided additional information on the epidemiology of SLE. The incidence and prevalence of SLE varies considerably across the countries. The burden of the disease is considerably elevated among non-white racial groups. There is a trend towards higher incidence and prevalence of SLE in Europe and Australia compared to the U.S.A. In Europe, the highest prevalence was reported in Sweden, Iceland and Spain. There are marked disparities in SLE rates worldwide. This variability may reflect true differences across populations, or result from methodological differences of studies. The true geographic, racial, and temporal differences in SLE incidence and prevalence may yield important clues to the etiology of disease.
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          Incidence of systemic lupus erythematosus. Race and gender differences.

          To examine racial differences in the incidence of systemic lupus erythematosus (SLE). A population-based registry of SLE patients in Allegheny County, Pennsylvania, was used to identify incident cases of SLE diagnosed between January 1, 1985 and December 31, 1990, from 3 sources, by medical record review (University of Pittsburgh Lupus Databank, rheumatologists, and hospitals). Capture-recapture methods using log-linear models were used to estimate the level of case-finding and to calculate 95% confidence intervals (CI). Incidence rates were calculated per 100,000 population. A total of 191 definite and 78 probable incident cases of SLE were identified, and the overall annual incidence rates were 2.4 (95% CI 2.1-2.8) and 1.0 (95% CI 0.8-1.3), respectively. The crude incidence rates of definite SLE were 0.4 for white males, 3.5 for white females, 0.7 for African-American males, and 9.2 for African-American females. The annual incidence rates of definite SLE remained fairly constant over the study interval. African-American females with definite SLE had a younger mean age at diagnosis compared with white females (P < 0.05). Since the overall ascertainment rate was high (85%; 95% CI 78-92%), the ascertainment-corrected incidence rate for definite SLE, 2.8 (95% CI 2.6-3.2), was similar to the crude rate. Our rates clearly confirm previous reports of an excess incidence of SLE among females compared with males and among African-Americans compared with whites. We have used capture-recapture methods to improve the accuracy of SLE incidence rates, and we advocate their use to facilitate comparisons across studies.
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            Author and article information

            Affiliations
            [* ]University of Maryland Medical Center, Baltimore, Maryland
            []University of Maryland School of Medicine, Department of Emergency Medicine, Baltimore, Maryland
            Author notes
            Address for Correspondence: Zachary DW Dezman, MD, MS, University of Maryland School of Medicine,110 Paca Street, Suite 200, 6th floor, Baltimore, MD 21201. Email: zdezman@ 123456em.umaryland.org .
            Journal
            Clin Pract Cases Emerg Med
            Clin Pract Cases Emerg Med
            Clinical Practice and Cases in Emergency Medicine
            University of California Irvine, Department of Emergency Medicine publishing Western Journal of Emergency Medicine
            2474-252X
            May 2017
            21 March 2017
            : 1
            : 2
            : 76-80
            5965421
            10.5811/cpcem.2017.3.34163
            cpcem-01-76
            © 2017 Dezman et al.

            This is an open access article distributed in accordance with the terms of the Creative Commons Attribution (CC BY 4.0) License. See: http://creativecommons.org/licenses/by/4.0/

            Categories
            Clinicopathological Cases from the University of Maryland

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