Systemic Lupus Erythematosus: Diagnosis and Treatment  

Diagnosis

The American College of Rheumatology has established clinical and laboratory criteria to aid in diagnosis. At least 4 of the following 11 criteria should be present for diagnosis:

• Malar rash
• Discoid rash
• Photosensitivity
• Oral or nasopharyngeal ulcers
• Arthritis in more than 2 joints(in 90% of cases)
• Serositis (pleuritis or pleural effusion, pericarditis or pericardial effusion)
• Renal disorders (proteinuria)
• Neurologic disorders (seizure, psychosis)
• Hematologic disorders (anemia, leukopenia, thrombocytopenia)
• Immunologic disorders (anti–DNA antibodies, anti–Smith antibodies, antiphospholipid antibodies, false–positive syphilis serology)
• Antinuclear antibodies (ANA) (in 98% of cases)

Treatment

Dietary changes, physical activity, smoking cessation, and avoidance of sun exposure are proven nonmedical measures for managing SLE. Pregnancy should be avoided during active disease, due to a high risk of miscarriage and maternal complications. Medical therapy is tailored to specific organ involvement.

Nonsteroidal anti–inflammatory drugs (NSAIDs) are used in patients with arthritis, myalgias, fever, and mild serositis. However, sulfa–containing NSAIDs (celecoxib) should be used with caution, as they may exacerbate the disease. The risk of adverse events associated with NSAIDs, especially COX–2 inhibitors, should be considered when prescribing these medications.

Steroids are frequently used during exacerbations and are particularly effective for pericarditis, nephritis, cerebritis, and arthritis. It is often wise to seek steroid–sparing therapy so as to mitigate the long–term adverse effects of glucocorticoids. Because long–term steroid use can affect bone structure, dual energy x–ray absortiometry (DEXA) scanning may be helpful to monitor skeletal integrity.

Disease–modifying antirheumatic drugs (azathioprine, hydroxychloroquine, mycophenolate mofetil, cyclophosphamide) may be useful in cases refractory to NSAIDs and/or steroids. In particular, hydroxychloroquine is often beneficial for skin and musculoskeletal symptoms, and mycophenolate mofetil appears to have renal–protective effects. Cyclophosphamide is often used in situations of major organ involvement and life–threatening disease.

Experimental therapies under investigation include stem cell transplantation, anti–B–cell antibodies, other anticytokine antibodies, intravenous immune globulin, and thalidomide.

A number of drugs have reportedly exacerbated the disease or resulted in drug allergy. In particular, sulfonamides should be avoided, as they may exacerbate symptoms. There are also anecdotal reports of aseptic meningitis developing during NSAID use in SLE, such as with ibuprofen.

Measures to protect against direct and indirect sunlight exposure should be emphasized, including daily use of high–SPF sunscreen, wearing long–sleeved shirts and wide–brimmed hats, and avoiding midday sun.

Low–impact, weight–bearing exercise is important for maintaining cardiovascular and bone health in SLE patients.