Systemic Lupus Erythematosus: Practice Essentials

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

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