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. See the image below. Cutaneous Clues to Accurately Diagnosing Rheumatologic Disease, a Critical Images slideshow, to help recognize cutaneous manifestations of rheumatologic diseases. 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. 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 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 level 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 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: Antimalarials (eg, hydroxychloroquine) Corticosteroids (eg, methylprednisolone, prednisone), short-term use recommended Nonbiologic DMARDS: Cyclophosphamide, methotrexate, azathioprine, mycophenolate, cyclosporine Nonsteroidal anti-inflammatory drugs (NSAIDS; eg, ibuprofen, naproxen, diclofenac) Biologic DMARDs (disease-modifying antirheumatic drugs): Belimumab, rituximab, IV immune globulin Source
