Derivation and Validation of Systemic Lupus International Collaborating Clinics Classification Criteria for Systemic Lupus Erythematosus
01 Oct 2011-
TL;DR: 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 as mentioned in this paper.
Abstract: OBJECTIVE
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.
METHODS
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.
RESULTS
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).
CONCLUSION
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.
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TL;DR: In this chapter, issues in classification and diagnosis of the various sub-types of CLE are discussed, as well as an update on therapeutic management.
Abstract: Cutaneous lupus erythematosus (CLE) encompasses a wide range of dermatologic manifestations, which may or may not be associated with the development of systemic disease. Cutaneous lupus is divided into several sub-types, including acute CLE (ACLE), sub-acute CLE (SCLE) and chronic CLE (CCLE). CCLE includes discoid lupus erythematosus (DLE), LE profundus (LEP), chilblain cutaneous lupus and lupus tumidus. The diagnosis of these diseases requires proper classification of the sub-type, through a combination of physical examination, laboratory studies, histology, antibody serology and occasionally direct immunofluorescence, while ensuring to exclude systemic disease. The treatment of cutaneous lupus consists of patient education on proper sun protection along with appropriate topical and systemic agents. Systemic agents are indicated in cases of widespread, scarring or treatment-refractory disease. In this chapter, we discuss issues in classification and diagnosis of the various sub-types of CLE, as well as provide an update on therapeutic management.
226 citations
TL;DR: In this article, the deficiency of early complement proteins from the Classical Pathway is strongly associated with development of systemic lupus erythematous (SLE) - mainly C1q deficiency (93%) and C4 deficiency (75%).
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194 citations
Johns Hopkins University School of Medicine1, Lund University2, Johns Hopkins University3, University of Alabama at Birmingham4, University of Birmingham5, Oklahoma Medical Research Foundation6, Laval University7, Central Manchester University Hospitals NHS Foundation Trust8, University of California, Los Angeles9, Northwestern University10, Hanyang University11, Dalhousie University12, University Health Network13, McGill University14, North Shore-LIJ Health System15, Allegheny General Hospital16, Toronto Western Hospital17, University of California, San Diego18, University of Pennsylvania19, Hairmyres Hospital20, University of the Basque Country21, St Thomas' Hospital22, University of Copenhagen23, New York University24, University of North Carolina at Chapel Hill25, Karolinska Institutet26, SUNY Downstate Medical Center27, University of Manitoba28, Wake Forest University29, University of Louisville30, Emory University31, Istanbul University32, Medical University of South Carolina33, University of Texas Health Science Center at San Antonio34, Cedars-Sinai Medical Center35, University of Maryland, Baltimore36
TL;DR: The usefulness of anti-C1q in SLE, especially in lupus nephritis, and in combination with anti-dsDNA and low complement was the strongest serological association with renal involvement is supported, independent of demographics and other serologies.
Abstract: Objective: Anti-C1q has been associated with systemic lupus erythematosus (SLE) and lupus nephritis in previous studies. We studied anti-C1q specificity for SLE (vs rheumatic disease controls) and the association with SLE manifestations in an international multicenter study. Methods: Information and blood samples were obtained in a cross-sectional study from patients with SLE (n ¼ 308) and other rheumatologic diseases (n ¼ 389) from 25 clinical sites (84% female, 68% Caucasian, 17% African descent, 8% Asian, 7% other). IgG anti-C1q against the collagen-like region was measured by ELISA. Results: Prevalence of anti-C1q was 28% (86/308) in patients with SLE and 13% (49/389) in controls (OR ¼ 2.7, 95% CI: 1.8–4, p < 0.001). Anti-C1q was associated with proteinuria (OR ¼ 3.0, 95% CI: 1.7–5.1, p < 0.001), red cell casts (OR ¼ 2.6, 95% CI: 1.2–5.4, p ¼ 0.015), anti-dsDNA (OR ¼ 3.4, 95% CI: 1.9–6.1, p < 0.001) and anti-Smith (OR ¼ 2.8, 95% CI: 1.5–5.0, p ¼ 0.01). Anti-C1q was independently associated with renal involvement after adjustment for demographics, ANA, anti-dsDNA and low complement (OR ¼ 2.3, 95% CI: 1.3–4.2, p < 0.01). Simultaneously positive anti-C1q, anti-dsDNA and low complement was strongly associated with renal involvement (OR ¼ 14.9, 95% CI: 5.8–38.4, p < 0.01). Conclusions: Anti-C1q was more common in
101 citations
University of Paris1, French Institute of Health and Medical Research2, Katholieke Universiteit Leuven3, National Institutes of Health4, University of Padua5, University of Calgary6, Alberta Children's Hospital7, Nagoya University8, University of Guadalajara9, China-Japan Friendship Hospital10, Charles University in Prague11, University of Brescia12, University of Debrecen13
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TL;DR: This review attempts to delineate the performance of theCurrent sets of criteria, the reasons for the decision for classification, and not diagnostic, criteria, and to provide a background of the current approach taken.
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76 citations