THERAPY.

Treatment must be individualized for each patient. Not all patients require steroids; steroids have the potential of doing more harm than good. The goals for each therapy and the potential of each for benefit and risk should be considered carefully. The goal is to maintain organ function and prevent permanent organ injury. The threat of a chronic disease can be very stressful, as can visiting a physician frequently and having many laboratory tests--and waiting for the results. Thus emotional support is essential, as well as counseling and the provision of written (and other) material. Patients should be assured that SLE is mild in most patients, that it is rarely life threatening, and that serious organ involvement can usually be prevented. Support by family, friends, and organizations such as the Lupus Foundation of America and the Arthritis Foundation is often helpful.

It is important to determine whether the symptoms and signs are due to SLE or something else(Table 289-10). For instance, fever is more likely to be due to an infection and fatigue due to lack of sleep. Low complement levels, high anti-DNA levels, and/or high immune complex levels suggest active SLE.

Preventive measures are useful. Patients should avoid using sulfonamides, penicillin, and high-estrogen birth control pills, which may exacerbate the lupus. Exercise has been demonstrated to ameliorate the fatigue associated with SLE. Patients should be questioned regarding their degree of photosensitivity; not all patients have photosensitivity, and the degree may vary, including variation over time. Photosensitive patients should use sunscreens daily with an SPF of at least 15; for those who are very photosensitive, sunscreen should be used twice daily. Photosensitizing medications (e.g., tetracyclines, psoralens) should be avoided. In postmenopausal women, estrogen is recommended for its benefit regarding osteoporosis and coronary artery disease (women with SLE are at excess risk for these conditions), unless the patient has contraindications or the SLE relapses. Immunization with flu and pneumococcal vaccines is advisable.

In treating SLE one should consider which organ is involved and to what degree ("severity").Table 289-11provides an outline of therapy based on organ involvement; treatment starts conservatively and, if the response is inadequate, becomes more aggressive.

Treatment of lupus nephritis(Table 289-12)should be based on whether the disease is considered active, the type of nephritis (see earlier), and the severity. The goal of treatment should be to improve, maintain, and prevent deterioration in renal function. For mesangial or focal glomerulonephritis, bed rest or a short course of prednisone (30 mg/day) will usually suffice to clear the urinary and serologic abnormalities. For diffuse proliferative glomerulonephritis, more vigorous treatment is usually given. Patients are generally treated with 1 mg/kg of prednisone. If azotemia is present (especially if the creatinine level is greater than 1.2 mg/dL), an immunosuppressive should be added, either azathioprine in doses of 50 to 200 mg/day (a dose to achieve slight leukopenia) or cyclophosphamide. Pulse steroids (1 g of IV methylprednisolone per day for 1 to 3 doses) may be useful acutely until the immunosuppressives start working (which may be 7 to 10 days). Cyclophosphamide given intravenously (in the morning) once monthly (for 6 months) and then every 3 months (for eight doses) appears to be as effective, and less toxic than the same drug given by mouth. The risks of this therapy (malignancy, infections, hair loss, infertility) should be discussed with patients. The initial dose is 0.85 g/1.7 m²body surface area. The WBC count is determined 7 to 10 days later, and the next dose is adjusted (to a maximum of 2 g/1.7 m²) to achieve

a WBC count of about 4000. Acute membranous glomerulonephritis usually responds to high doses of prednisone (1 mg/kg of prednisone per day) or pulse steroids. If not, a trial of immunosuppressives should be instituted. Hypertension should be treated vigorously; angiotensin-converting enzyme inhibitors appear to help proteinuria. Diuretics are useful to control edema and hypertension. There is no evidence that plasmapheresis benefits the management of lupus nephritis. For active renal disease, patients should be monitored once a week with urinalysis, serum creatinine determination, and immune function tests (complement, anti-DNA). When the disease becomes inactive, monitoring will be less frequent, depending on the degree of residual damage--patients with nephrotic-range proteinuria or those with azotemia need to be monitored more closely.