Acute neuropsychiatric lupus should be treated aggressively and quickly in the hope of reversing the process, which usually means high doses of prednisone (1 to 2 mg/kg), as well as antipsychotics. Once the psychosis has cleared, the dosage of steroids should be tapered rapidly because patients are at high risk for infection; adding immunosuppressives, particularly cyclophosphamide, may be beneficial. Steroids themselves may induce psychosis; therefore it is important to serially monitor the patient with objective measures, including EEG, MRI, and some antibrain antibodies, as well as CSF protein. However, often one must rely on clinical judgment. Seizure disorders are treated with anticonvulsants (e.g., phenytoin, phenobarbital, carbamazepine); no evidence exists that these medications exacerbate SLE. Multiple small strokes may be due to antiphospholipid antibodies.

Treatment of the antiphospholipid antibody syndrome remains controversial. Low levels of this antibody rarely cause symptoms. Patients with high levels and no symptoms should be treated with low-dose aspirin (81 mg/day); with symptoms, chronic warfarin (Coumadin) therapy is used at a dosage to maintain an International Normalized Ratio between 3 and 4.

Patients started on prednisone therapy should receive high doses only until the inflammation has subsided--thus the patient should be assessed frequently regarding specific organ function, as well as immune status (complement, anti-DNA antibodies). For acute, severe lupus, split doses are recommended, then a switch to a daily morning dose. For long-term management, the benefit-risk issue needs to be discussed. The prednisone dosage should then be tapered, the rate depending on the severity of organ inflammation and damage, the maximum dose, and side effects from prednisone (i.e., psychological changes, insomnia, weight gain, hypertension, diabetes, peptic ulcer, infections such as acne, cushingoid features, adrenal suppression, osteonecrosis, myopathy, impaired wound and fracture healing, skin atrophy, cataracts, atherosclerosis, growth retardation). Once a dose of about 10 to 20 mg/day is achieved and the disease is "quiet," the patient can start every-other-day prednisone therapy by decreasing the dosage progressively on alternate days. Patients receiving long-term steroids should be monitored for osteoporosis and treated aggressively for it with either estrogen, calcium, vitamin D, bisphosphonates, or combinations thereof. Patients taking antimalarials should have an ophthalmologic examination every 6 months; those taking NSAIDs should be watched for gastrointestinal and renal toxicity.

PROGNOSIS.

The prognosis for SLE patients in theUnited Stateshas improved dramatically since the 1950s, when the survival rate was approximately 50% at 5 years; in 1994, it was approximately 90% at 10 years. The prognosis is worse for those with CNS involvement, hypertension, azotemia, and early age of onset. The major cause of death is infection. Although there is an impression of greater awareness of the disease, its clinical expression has not changed in 20 years; nor is there evidence that it is being diagnosed earlier.

Reference

Cervera R, Khamashta MA, Font J, et al: Systemic lupus erythematosus: Clinical and immunologic patterns of disease expression in a cohort of 1000 patients. Medicine (Baltimore) 72:113, 1993.The largest cohort described in a cooperative European anthology--percentage of clinical and immunologic features.

Hahn B, Wallace D (eds): Dubois' Lupus Erythematosus.Malvern,PA, Lea & Febiger, 1993.An excellent source.

Lahita RG (ed): Systemic Lupus Erythematosus.New York, Churchill Livingstone, 1992.A comprehensive series of chapters on both mechanism and treatment arranged by organ systems.

Schur PH (ed): The Clinical Management of Systemic Lupus Erythematosus, 2nd ed.Philadelphia, JB Lippincott, 1996.A book for internists and generalists on practical management of SLE patients.