Systemic lupus erythematosus (sle)

8/28/14 Sy stemic Lupus Ery thematosus (SLE)
1/12emedicine.medscape.com/article/332244-ov erv iew
Practice Essentials
Systemic lupus erythematosus (SLE) is a chronic inflammatory disease that has protean manifestations and
follows a relapsing and remitting course. More than 90% of cases of SLE occur in women, frequently starting at
childbearing age.
Signs and symptoms
SLE is a chronic autoimmune disease that can affect almost any organ system; thus, its presentation and course
are highly variable, ranging from indolent to fulminant.
In childhood-onset SLE, there are several clinical symptoms more commonly found than in adults, including malar
rash, 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 suspicion
of 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 the
diagnostic criteria helps clinicians to recognize SLE and to subclassify this complex disease based on the pattern
of 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 SLE
classification criteria in 2012, they classified a person as having SLE in the presence of biopsy-proven lupus
nephritis 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:
Serositis
Oral ulcers
Arthritis
Photosensitivity
Blood disorders
Renal involvement
Antinuclear antibodies
Immunologic phenomena (eg, dsDNA; anti-Smith [Sm] antibodies)
Neurologic disorder
Malar rash
Discoid rash
Testing
The following are useful standard laboratory studies when SLE is suspected:
CBC with differential
Serum creatinine
Urinalysis with microscopy
Other laboratory tests that may be used in the diagnosis of SLE are as follows:
ESR or CRP results
Complement levels
Liver function tests
Creatine kinase assay
Spot protein/spot creatinine ratio
Autoantibody tests
Imaging studies
The following imaging studies may be used to evaluate patients with suspected SLE:
Joint radiography
Chest radiography and chest CT scanning
Echocardiography
Brain MRI/ MRA
Cardiac MRI
Procedures
Procedures that may be performed in patients with suspected SLE include the following:
Arthrocentesis
Lumbar puncture
Renal 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 globulin
Nonbiologic DMARDS: Cyclophosphamide, methotrexate, azathioprine, mycophenolate, cyclosporine
Nonsteroidal 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 butterfly rash, w ith distribution over the cheeks and nasal bridge. Note that the fixed 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 Disclosures
Author
Christie M Bartels, MD, MS Assistant Professor of Rheumatology, Department of Medicine, University of
Wisconsin 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 Medical
Association
Chief Editor
Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New
York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania
Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College
of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Additional Contributors
Gino A Farina, MD, FACEP, FAAEM Associate Professor of Clinical Emergency Medicine, Albert Einstein
College 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 of
Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency
Medicine
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

Physicians, and American College of Rheumatology
Julie Hildebrand, MD Consulting Staff, Department of Internal Medicine, Associated Physicians of Madison,
WI
Richard S Krause, MD Senior Clinical Faculty/Clinical Assistant Professor, Department of Emergency
Medicine, 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, American
Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic
Emergency Medicine
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 Emergency
Medicine and American College of Emergency Physicians
Mark J Leber, MD, MPH Assistant Professor of Emergency Medicine in Clinical Medicine, Weill Cornell
Medical 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 Emergency
Physicians and American College of Physicians
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 and
American 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 Center
College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Medscape Salary Employment
Anuritha Tirumani, MD Research Coordinator, Department of Emergency Medicine, Brooklyn Hospital Center
Acknowledgements
The authors would like to thank Joanna Wong for assistance in preparation of revisions to this topic.
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