systemic lupus erythematosus (sle)
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8/28/14 Sy stemic Lupus Ery thematosus (SLE)
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Practice Essentials
Systemic lupus erythematosus (SLE) is a chronic inflammatory disease that has protean manifestations andfollows a relapsing and remitting course. More than 90% of cases of SLE occur in women, frequently starting atchildbearing age.
Signs and symptoms
SLE is a chronic autoimmune disease that can affect almost any organ system; thus, its presentation and courseare highly variable, ranging from indolent to fulminant.
In childhood-onset SLE, there are several clinical symptoms more commonly found than in adults, including malarrash, ulcers/mucocutaneous involvement, renal involvement, proteinuria, urinary cellular casts, seizures,
thrombocytopenia, hemolytic anemia, fever, and lymphadenopathy.[1]
In adults, Raynaud pleuritis and sicca are twice as common as in children and adolescents.[1]
The classic presentation of a triad of fever, joint pain, and rash in a woman of childbearing age should prompt
investigation into the diagnosis of SLE.[2, 3]
Patients may present with any of the following manifestations[4] :
Constitutional (eg, fatigue, fever, arthralgia, weight changes)Musculoskeletal (eg, arthralgia, arthropathy, myalgia, frank arthritis, avascular necrosis)Dermatologic (eg, malar rash, photosensitivity, discoid lupus)Renal (eg, acute or chronic renal failure, acute nephritic disease)Neuropsychiatric (eg, seizure, psychosis)Pulmonary (eg, pleurisy, pleural effusion, pneumonitis, pulmonary hypertension, interstitial lung disease)Gastrointestinal (eg, nausea, dyspepsia, abdominal pain)Cardiac (eg, pericarditis, myocarditis)Hematologic (eg, cytopenias such as leukopenia, lymphopenia, anemia, or thrombocytopenia)
In patients with suggestive clinical findings, a family history of autoimmune disease should raise further suspicionof SLE.
See Clinical Presentation for more detail.
Diagnosis
The diagnosis of SLE is based on a combination of clinical findings and laboratory evidence. Familiarity with thediagnostic criteria helps clinicians to recognize SLE and to subclassify this complex disease based on the patternof target-organ manifestations.
The presence of 4 of the 11 American College of Rheumatology (ACR) criteria yields a sensitivity of 85% and a
specificity of 95% for SLE.[5, 6]
When the Systemic Lupus International Collaborating Clinics (SLICC) group revised and validated the ACR SLEclassification criteria in 2012, they classified a person as having SLE in the presence of biopsy-proven lupusnephritis with ANA or anti-dsDNA antibodies or if 4 of the diagnostic criteria, including at least 1 clinical and 1
immunologic criterion, have been satisfied.[7]
ACR mnemonic of SLE diagnostic criteria
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The following are the ACR diagnostic criteria in SLE, presented in the "SOAP BRAIN MD" mnemonic:
SerositisOral ulcersArthritisPhotosensitivityBlood disordersRenal involvementAntinuclear antibodiesImmunologic phenomena (eg, dsDNA; anti-Smith [Sm] antibodies)Neurologic disorderMalar rashDiscoid rash
Testing
The following are useful standard laboratory studies when SLE is suspected:
CBC with differentialSerum creatinineUrinalysis with microscopy
Other laboratory tests that may be used in the diagnosis of SLE are as follows:
ESR or CRP resultsComplement levelsLiver function testsCreatine kinase assaySpot protein/spot creatinine ratioAutoantibody tests
Imaging studies
The following imaging studies may be used to evaluate patients with suspected SLE:
Joint radiographyChest radiography and chest CT scanningEchocardiographyBrain MRI/ MRACardiac MRI
Procedures
Procedures that may be performed in patients with suspected SLE include the following:
ArthrocentesisLumbar punctureRenal biopsy
See Workup for more detail.
Management
Management of SLE often depends on the individual patient’s disease severity and disease manifestations,[8]
although hydroxychloroquine has a central role for long-term treatment in all SLE patients.
Pharmacotherapy
Medications used to treat SLE manifestations include the following:
Biologic DMARDs (disease-modifying antirheumatic drugs): Belimumab, rituximab, IV immune globulinNonbiologic DMARDS: Cyclophosphamide, methotrexate, azathioprine, mycophenolate, cyclosporineNonsteroidal anti-inflammatory drugs (NSAIDS; eg, ibuprofen, naproxen, diclofenac)Corticosteroids (eg, methylprednisolone, prednisone)
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Antimalarials (eg, hydroxychloroquine)
See Treatment and Medication for more detail.
Image library
The classic malar rash, also know n as a butterf ly rash, w ith distribution over the cheeks and nasal bridge. Note that the f ixed erythema,
sometimes w ith mild induration as seen here, characteristically spares the nasolabial folds.
Systemic Lupus Erythematosus (SLE)
Author: Christie M Bartels, MD, MS; Chief Editor: Herbert S Diamond, MD more...
Updated: Feb 19, 2014
Contributor Information and DisclosuresAuthorChristie M Bartels, MD, MS Assistant Professor of Rheumatology, Department of Medicine, University ofWisconsin School of Medicine and Public Health
Christie M Bartels, MD, MS is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and American College of Rheumatology
Disclosure: Nothing to disclose.
Coauthor(s)Daniel Muller, MD, PhD Associate Professor of Medicine, Department of Medicine, Section of Rheumatology,University of Wisconsin School of Medicine and Public Health
Daniel Muller, MD, PhD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American College of Rheumatology, and American Holistic MedicalAssociation
Disclosure: Nothing to disclose.
Chief EditorHerbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of NewYork Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western PennsylvaniaHospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American Collegeof Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: Nothing to disclose.
Additional ContributorsGino A Farina, MD, FACEP, FAAEM Associate Professor of Clinical Emergency Medicine, Albert EinsteinCollege of Medicine; Program Director, Department of Emergency Medicine, Long Island Jewish Medical Center
Gino A Farina, MD, FACEP, FAAEM is a member of the following medical societies: American Academy ofEmergency Medicine, American College of Emergency Physicians, and Society for Academic EmergencyMedicine
Disclosure: Nothing to disclose.
Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine,Temple University School of Medicine
Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of
8/28/14 Sy stemic Lupus Ery thematosus (SLE)
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Physicians, and American College of Rheumatology
Disclosure: Nothing to disclose.
Julie Hildebrand, MD Consulting Staff, Department of Internal Medicine, Associated Physicians of Madison,WI
Disclosure: Nothing to disclose.
Richard S Krause, MD Senior Clinical Faculty/Clinical Assistant Professor, Department of EmergencyMedicine, University of Buffalo State University of New York School of Medicine and Biomedical Sciences
Richard S Krause, MD is a member of the following medical societies: Alpha Omega Alpha, AmericanAcademy of Emergency Medicine, American College of Emergency Physicians, and Society for AcademicEmergency Medicine
Disclosure: Nothing to disclose.
Viraj S Lakdawala, MD Clinical Instructor of Emergency Medicine, University of California, San Francisco,School of Medicine; Attending Physician, San Francisco General Hospital
Viraj S Lakdawala, MD is a member of the following medical societies: American Academy of EmergencyMedicine and American College of Emergency Physicians
Disclosure: Nothing to disclose.
Mark J Leber, MD, MPH Assistant Professor of Emergency Medicine in Clinical Medicine, Weill CornellMedical College; Attending Physician, Lincoln Medical and Mental Health Center
Mark J Leber, MD, MPH is a member of the following medical societies: American College of EmergencyPhysicians and American College of Physicians
Disclosure: Nothing to disclose.
Carlos J Lozada, MD Director of Rheumatology Fellowship Program, Professor, Department of Medicine,Division of Rheumatology and Immunology, University of Miami, Leonard M Miller School of Medicine
Carlos J Lozada, MD is a member of the following medical societies: American College of Physicians andAmerican College of Rheumatology
Disclosure: Pfizer Honoraria Speaking and teaching; Amgen Honoraria Speaking and teaching
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical CenterCollege of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Medscape Salary Employment
Anuritha Tirumani, MD Research Coordinator, Department of Emergency Medicine, Brooklyn Hospital Center
Disclosure: Nothing to disclose.
Acknowledgements
The authors would like to thank Joanna Wong for assistance in preparation of revisions to this topic.
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