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Systemic Lupus Erythematosus (SLE)

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General Information

Description

  • systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder of connective tissue characterized by autoantibodies that target nuclear antigens, remissions and flares, and highly variable clinical presentation, disease course, and prognosis1,2,3,4,5

Definitions

  • disease severity
    • mild disease1
      • constitutional symptoms
      • mild arthritis
      • rash affecting ≤ 9% of body surface area
      • platelet count 50-100 x 103/mm3
      • SLEDAI score ≤ 6
      • British Isles Lupus Assessment Group (BILAG) index Grade C or ≤ 1 BILAG Grade B manifestation
    • moderate disease1
      • rheumatoid arthritis-like arthritis
      • rash affecting 9%-18% of body surface area
      • cutaneous vasculitis affecting ≤ 18% of body surface area
      • platelet count 20-50 x 103/mm3
      • serositis
      • SLEDAI score 7-12
      • ≥ 2 BILAG Grade B manifestations
    • severe disease1
      • major organ-threatening disease, such as nephritis, cerebritis, myelitis, pneumonitis, mesenteric vasculitis
      • thrombocytopenia with platelet count < 20 x 103/mm3
      • thrombotic thrombocytopenic purpura-like disease or acute hemophagocytic syndrome
      • SLEDAI score > 12
      • ≥ 1 BILAG Grade A manifestations
  • disease activity
    • active SLE - presence of symptoms, signs, or test results that2
      • can be attributed to active inflammation
      • indicate organ involvement that can be reversed with therapy
    • low disease activity1
      • in patients on antimalarials - SLE Disease Activity Index (SLEDAI) score ≤ 3
      • in patients receiving ≤ 7.5 mg prednisone and well-tolerated immunosuppressant agents
        • SLEDAI score ≤ 4
        • physician global assessment (PGA) score ≤ 1
    • disease flare - measurable increase in disease activity usually leading to change of treatment1
    • remission in patients with lupus nephritis1
      • partial remission defined as both of
        • ≥ 50% reduction in proteinuria to subnephrotic levels
        • serum creatinine within 10% from baseline
      • complete renal remission defined as both of
        • proteinuria < 500 mg/24 hours
        • serum creatinine within 10% from baseline
    • complete remission - absence of clinical activity without use of glucocorticoids or immunosuppressant drugs1

References

General references used

  1. Fanouriakis A, Kostopoulou M, Alunno A, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019 Jun;78(6):736-745OpenInNew
  2. Mosca M, Tani C, Aringer M, et al. European League Against Rheumatism recommendations for monitoring patients with systemic lupus erythematosus in clinical practice and in observational studies. Ann Rheum Dis. 2010 Jul;69(7):1269-74OpenInNewfull-textOpenInNew
  3. Fava A, Petri M. Systemic lupus erythematosus: Diagnosis and clinical management. J Autoimmun. 2019 Jan;96:1-13OpenInNew
  4. Oku K, Atsumi T. Systemic lupus erythematosus: nothing stale her infinite variety. Mod Rheumatol. 2018 Sep;28(5);758-765OpenInNew
  5. Gergianaki I, Bortoluzzi A, Bertsias G. Update on the epidemiology, risk factors, and disease outcomes of systemic lupus erythematosus. Best Pract Res Clin Rheumatol. 2018 Apr;32(2);188-205OpenInNew

Recommendation grading systems used

  • European League Against Rheumatism (EULAR) guideline grading system
    • Oxford Centre for Evidence Based Medicine (CEBM)OpenInNew classifications used in EULAR recommendations
      • CEBM grades of recommendation
        • Grade A - consistent level 1 studies
        • Grade B - consistent level 2 or 3 studies or extrapolations from level 1 studies
        • Grade C - level 4 studies or extrapolations from level 2 or 3 studies
        • Grade D - level 5 evidence or troublingly inconsistent or inconclusive studies of any level
      • CEBM levels of evidence
        • Level 1a - systematic review with homogeneity of randomized controlled trials (RCTs)
        • Level 1b - individual RCT with narrow confidence interval
        • Level 1c - all or none case series
        • Level 2a - systematic review with homogeneity of cohort studies
        • Level 2b - individual cohort study or low-quality RCT
        • Level 2c - "outcomes" research, ecological studies
        • Level 3a - systematic review with homogeneity of case-control studies
        • Level 3b - individual case-control study
        • Level 4 - case series, poor-quality cohort and case-control studies
        • Level 5 - expert opinion without explicit critical appraisal, or based on physiology, bench research or "first principles"
    • Reference - EULAR recommendations on monitoring patients with SLE in clinical practice and in observational studies (19892750Ann Rheum Dis 2010 Jul;69(7):1269OpenInNew)
  • European League Against Rheumatism (EULAR) 2010 guideline grading system for management of SLE with neuropsychiatric manifestations
    • strength of statement
      • Grade A - based on category 1 studies
      • Grade B - based on category 2 evidence or extrapolated recommendations from category 1 evidence
      • Grade C - based on category 3 evidence or extrapolated recommendations from category 2 evidence
      • Grade D - expert opinion or standard of care
    • category of evidence
      • diagnostic or prognostic studies
        • Category 1 - available evidence is strong and includes consistent results from well-designed, well-conducted studies
        • Category 2 - available evidence is sufficient to determine effects, but confidence in estimate is constrained by factors including number, size, or quality of individual studies, inconsistency of findings across individual studies, or limited generalizability of findings
        • Category 3 - available evidence is insufficient due to limited number or size of studies, important flaws in study design or methods, inconsistency of findings across individual studies, gaps in chain of evidence, or lack of information on important outcomes
      • intervention studies
        • Category 1 - based on ≥ 1 randomized controlled trial (RCT) or meta-analysis of RCTs
        • Category 2 - based on controlled (nonrandomized) studies
        • Category 3 - based on descriptive studies, such as comparative studies, correlation studies, or case-control studies
    • Reference - EULAR 2010 guideline on management of SLE with neuropsychiatric manifestations (Ann Rheum Dis 2010 Dec;69(12):2074OpenInNew)
  • European League Against Rheumatism (EULAR) 2019 guideline grading system
    • grades of recommendation
      • Grade A - consistent level 1 studies
      • Grade B - consistent level 2 or 3 studies or extrapolations from level 1 studies
      • Grade C - level 4 studies or extrapolations from level 2 or 3 studies
      • Grade D - level 5 evidence or troublingly inconsistent or inconclusive studies of any level
    • levels of evidence
      • for therapy/prevention/etiology/harm
        • Level 1a - systematic reviews of randomized controlled trials (RCTs)
        • Level 1b - individual, high-quality RCTs
        • Level 2a - systematic reviews of cohort studies
        • Level 2b - cohort study or low-quality RCT
        • Level 2c - "outcomes" research studies
        • Level 3a - systematic review of case-control studies
        • Level 3b - case-control studies
        • Level 4 - case series, poor-quality cohort and case-control studies
        • Level 5 - expert opinion
      • for risk factors/prognosis
        • Level 1a - systematic review of inception cohort studies
        • Level 1b - individual, high-quality inception cohort study
        • Level 2a - systematic review of retrospective cohort studies or data from RCT
        • Level 2b - retrospective cohort study or data from RCT
        • Level 2c - "outcomes" research studies
        • Level 4 - case series, poor-quality cohort and case-control studies
        • Level 5 - expert opinion
    • Reference - EULAR 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus (Ann Rheum Dis 2019 Jun;78(6):736OpenInNew)

Synthesized Recommendation Grading System for DynaMed

  • DynaMed systematically monitors clinical evidence to continuously provide a synthesis of the most valid relevant evidence to support clinical decision-making (see 7-Step Evidence-Based MethodologyOpenInNew).
  • Guideline recommendations summarized in the body of a DynaMed topic are provided with the recommendation grading system used in the original guideline(s), and allow DynaMed users to quickly see where guidelines agree and where guidelines differ from each other and from the current evidence.
  • In DynaMed (DM), we synthesize the current evidence, current guidelines from leading authorities, and clinical expertise to provide recommendations to support clinical decision-making in the Overview & Recommendations section.
  • We use the Grading of Recommendations Assessment, Development and Evaluation (GRADE)OpenInNew to classify synthesized recommendations as Strong or Weak.
    • Strong recommendations are used when, based on the available evidence, clinicians (without conflicts of interest) consistently have a high degree of confidence that the desirable consequences (health benefits, decreased costs and burdens) outweigh the undesirable consequences (harms, costs, burdens).
    • Weak recommendations are used when, based on the available evidence, clinicians believe that desirable and undesirable consequences are finely balanced, or appreciable uncertainty exists about the magnitude of expected consequences (benefits and harms). Weak recommendations are used when clinicians disagree in judgments of relative benefit and harm, or have limited confidence in their judgments. Weak recommendations are also used when the range of patient values and preferences suggests that informed patients are likely to make different choices.
  • DynaMed (DM) synthesized recommendations (in the Overview & Recommendations section) are determined with a systematic methodology:
    • Recommendations are initially drafted by clinical editors (including ≥ 1 with methodological expertise and ≥ 1 with content domain expertise) aware of the best current evidence for benefits and harms, and the recommendations from guidelines.
    • Recommendations are phrased to match the strength of recommendation. Strong recommendations use "should do" phrasing, or phrasing implying an expectation to perform the recommended action for most patients. Weak recommendations use "consider" or "suggested" phrasing.
    • Recommendations are explicitly labeled as Strong recommendations or Weak recommendations when a qualified group has explicitly deliberated on making such a recommendation. Group deliberation may occur during guideline development. When group deliberation occurs through DynaMed-initiated groups:
      • Clinical questions will be formulated using the PICO (Population, Intervention, Comparison, Outcome) framework for all outcomes of interest specific to the recommendation to be developed.
      • Systematic searches will be conducted for any clinical questions where systematic searches were not already completed through DynaMed content development.
      • Evidence will be summarized for recommendation panel review including for each outcome, the relative importance of the outcome, the estimated effects comparing intervention and comparison, the sample size, and the overall quality rating for the body of evidence.
      • Recommendation panel members will be selected to include at least 3 members that together have sufficient clinical expertise for the subject(s) pertinent to the recommendation, methodological expertise for the evidence being considered, and experience with guideline development.
      • All recommendation panel members must disclose any potential conflicts of interest (professional, intellectual, and financial), and will not be included for the specific panel if a significant conflict exists for the recommendation in question.
      • Panel members will make Strong recommendations if and only if there is consistent agreement in a high confidence in the likelihood that desirable consequences outweigh undesirable consequences across the majority of expected patient values and preferences. Panel members will make Weak recommendations if there is limited confidence (or inconsistent assessment or dissenting opinions) that desirable consequences outweigh undesirable consequences across the majority of expected patient values and preferences. No recommendation will be made if there is insufficient confidence to make a recommendation.
      • All steps in this process (including evidence summaries which were shared with the panel, and identification of panel members) will be transparent and accessible in support of the recommendation.
    • Recommendations are verified by ≥ 1 editor with methodological expertise, not involved in recommendation drafting or development, with explicit confirmation that Strong recommendations are adequately supported.
    • Recommendations are published only after consensus is established with agreement in phrasing and strength of recommendation by all editors.
    • If consensus cannot be reached then the recommendation can be published with a notation of "dissenting commentary" and the dissenting commentary is included in the topic details.
    • If recommendations are questioned during peer review or post publication by a qualified individual, or reevaluation is warranted based on new information detected through systematic literature surveillance, the recommendation is subject to additional internal review.

DynaMed Editorial Process

Special acknowledgements

  • DynaMed topics are written and edited through the collaborative efforts of the above individuals. Deputy Editors, Section Editors, and Topic Editors are active in clinical or academic medical practice. Recommendations Editors are actively involved in development and/or evaluation of guidelines.
  • Editorial Team role definitions
    Topic Editors define the scope and focus of each topic by formulating a set of clinical questions and suggesting important guidelines, clinical trials, and other data to be addressed within each topic. Topic Editors also serve as consultants for the internal DynaMed Editorial Team during the writing and editing process, and review the final topic drafts prior to publication.
    Section Editors have similar responsibilities to Topic Editors but have a broader role that includes the review of multiple topics, oversight of Topic Editors, and systematic surveillance of the medical literature.
    Recommendations Editors provide explicit review of DynaMed Overview and Recommendations sections to ensure that all recommendations are sound, supported, and evidence-based. This process is described in "Synthesized Recommendation Grading."
    Deputy Editors are employees of DynaMed and oversee DynaMed internal publishing groups. Each is responsible for all content published within that group, including supervising topic development at all stages of the writing and editing process, final review of all topics prior to publication, and direction of an internal team.
Choosing Wisely Canada acknowledges dissemination of their recommendations through DynaMed Plus to reach the point of clinical decision-making.

How to cite

National Library of Medicine, or "Vancouver style" (International Committee of Medical Journal Editors):

  • DynaMed [Internet]. Ipswich (MA): EBSCO Information Services. 1995 - . Record No. T115873, Systemic Lupus Erythematosus (SLE); [updated 2018 Nov 30, cited place cited date here]. Available from https://www.dynamed.com/topics/dmp~AN~T115873. Registration and login required.
  • KeyboardArrowRight

    Overview and Recommendations

    • Background

    • Evaluation

    • Management

  • Related Summaries

  • KeyboardArrowRight

    General Information

    • Description

    • Definitions

  • KeyboardArrowRight

    Epidemiology

    • Who is most affected

    • Incidence/Prevalence

    • KeyboardArrowRight

      Risk factors

      • Genetic risk factors

      • Lifestyle/Behavioral risk factors

      • Environmental risk factors

      • Health-related risk factors

      • Oral contraception and hormone replacement therapy

      • Factors associated with decreased risk

    • Associated conditions

  • KeyboardArrowRight

    Etiology and Pathogenesis

    • Causes

    • Pathogenesis

  • KeyboardArrowRight

    History and Physical

    • Clinical presentation

    • KeyboardArrowRight

      History

      • History of present illness (HPI)

      • Medication history

      • Family history (FH)

      • Social history (SH)

    • KeyboardArrowRight

      Physical

      • General physical

      • Skin

      • HEENT

      • Cardiac

      • Lungs

      • Abdomen

      • Extremities

      • Neuro

  • KeyboardArrowRight

    Diagnosis

    • Making the diagnosis

    • Differential diagnosis

    • Testing overview

    • Classification criteria

    • KeyboardArrowRight

      Blood tests

      • Autoantibodies

      • Other blood tests

    • Urine studies

    • Imaging studies

    • Other diagnostic testing

  • KeyboardArrowRight

    Management

    • Management overview

  • KeyboardArrowRight

    Complications and Prognosis

    • KeyboardArrowRight

      Complications

      • Lupus nephropathy

      • Cardiovascular disease (CVD)

      • Neurologic involvement (central, peripheral, or autonomic)

      • Hematologic abnormalities

      • Cancer

      • Other complications

    • KeyboardArrowRight

      Prognosis

      • Morbidity

      • Mortality

      • Effect of race on morbidity and mortality

  • Prevention and Screening

  • KeyboardArrowRight

    Quality Improvement

    • Choosing Wisely

    • Choosing Wisely Australia

    • Choosing Wisely Canada

  • KeyboardArrowRight

    Guidelines and Resources

    • KeyboardArrowRight

      Guidelines

      • International guidelines

      • United States guidelines

      • United Kingdom guidelines

      • Canadian guidelines

      • European guidelines

      • Asian guidelines

      • Central and South American guidelines

    • Review articles

    • MEDLINE search

  • Patient Information

  • KeyboardArrowRight

    ICD Codes

    • ICD-10 codes

  • KeyboardArrowRight

    References

    • General references used

    • Recommendation grading systems used

    • Synthesized Recommendation Grading System for DynaMed

    • DynaMed Editorial Process

    • Special acknowledgements

    • How to cite

Topic Editor
Jefferson R. Roberts MD
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Affiliations

Assistant Clinical Professor, University of Hawaii, John A. Burns School of Medicine; Hawaii, United States; Chief of Rheumatology Service and Chief of Medical Simulation, Tripler Army Medical Center; Hawaii, United States

Conflicts of Interest

Dr. Roberts declares no relevant financial conflicts of interest.

Recommendations Editor
Esther Jolanda van Zuuren MD
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Affiliations

Head of Allergy, Dermatology, and Venereology, Leiden University Medical Centre; Netherlands

Conflicts of Interest

Dr. van Zuuren declares no relevant financial conflicts of interest.

Deputy Editor
Vito Iacoviello MD, FIDSA
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Affiliations

Deputy Editor of Infectious Diseases, Immunology and Rheumatology, Dynamed; Massachusetts, United States; Assistant Professor of Medicine, Harvard Medical School; Massachusetts, United States; Chief of the Division of Infectious Diseases, Mount Auburn Hospital; Massachusetts, United States

Conflicts of Interest

Dr. Iacoviello declares no relevant financial conflicts of interest.

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