Medications

The medical treatment of juvenile SLE is complex, and should be highly individualized to each particular patient's needs. It is rare that a patient with juvenile SLE will not require medications at some point in the disease course, and most adolescents will require treatment with multiple immunosuppressant medications over long periods of time. Children and adolescents have more severe and active SLE than adult patients, and therefore studies have shown that they are treated with higher doses of corticosteroids and more likely to have had other immunosuppressants such as cyclophosphamide (13,14). Table 1 lists the common medications used in the treatment of adolescents with SLE.

The mainstay of treatment for active juvenile SLE is corticosteroids, given orally or intravenously when disease is quite active. High-dose (>1 mg/ kg/day) prednisone is generally effective in getting rapid disease control; a gradual tapering of the dose is required to prevent flaring. Nearly all adolescents who take corticosteroids will experience some side effects and toxicity, and these side effects are frequently very disturbing to the patients. The corticosteroid side effects which are most concerning to adolescents include increased acne, facial hair growth, and striae. Attention should be

Table 1 Medications Used in the Management of SLE in Adolescents

Nonsteroidal anti-inflammatory drugs

Control of musculoskeletal symptoms, i.e., arthritis Corticosteroids

Control of active disease

May require intravenous dosing to achieve rapid disease control, followed by oral dosing Split high-dose oral prednisone 1-2mg/kg/day recommended for rapid control of active disease, followed by a slow weaning. Schedule based on clinical and laboratory response Hydroxychloroquine

Adjunctive treatment with corticosteroids and immunosuppressives Control of mucocutaneous disease Immunosuppressives

Control of active disease and maintenance of quiescent disease Azathioprine Mycophenolate mofetil Cyclophosphamide Rituximab Anticoagulation

Prevention of recurrent thromboses in patients with high titer antiphospholipid antibodies who have had previous thrombosis Use of long-term low-dose aspirin to prevent thrombosis controversial directed to providing adequate treatment for the acne, with the involvement of a dermatologist if necessary. Facial hair growth should resolve as doses of steroid are tapered. Some patients develop severe widespread striae; this is not preventable, and there is no effective treatment for them once present. Infectious complications relating to steroids in combination with other immunosuppressants are frequent and require careful attention. Adolescents with SLE on steroids may be at increased risk for developing avascular necrosis. In some cases, there may be avascular necrosis in multiple sites. Careful evaluation should be done when an adolescent with SLE has persistent complaints of hip, knee, or other musculoskeletal pain that are not explained.

The immunosuppressant treatment for SLE will vary depending on the patient's clinical characteristics. Medications commonly used include aza-thioprine, mycophenolate mofetil, and intravenous cyclophosphamide (15). Many pediatric rheumatology centers will use intravenous cyclopho-sphamide for treating active lupus nephritis and other serious organ involvement of SLE (16). IV cyclophosphamide is generally given as a once monthly treatment for 6 to 7 months, with further treatments dependant on response. Among the many possible side effects of cyclophosphamide is thinning hair or significant hair loss; this is often very distressing to the patients.

The other important possible side effect of cyclophosphamide is impaired future fertility. Premature ovarian failure is a well-known possible complication of chemotherapy. In patients with SLE, the risk of developing premature ovarian failure after chemotherapy treatment seems to be lowest for children and adolescents. It is reported that 100% of those over age 30 years who receive cyclophosphamide will develop it, compared with about 50% of patients between ages 20 and 30 years and only 13% of patients younger than 20 years (17). However, this still is a significant concern for many patients. More recently, some centers have explored the possibility of using gonadotropin-releasing hormone analogs during the cyclophosphamide course to protect against premature ovarian failure. Somers et al. (18) showed a significant reduction in ovarian failure using this approach in a group of young adult women treated with monthly leuprolide acetate, a synthetic gonadotropin-releasing hormone analog.

It is very important, however, to stress with adolescents that they can still become pregnant while taking cyclophosphamide or afterwards—being on cyclophosphamide is not an effective form of contraception! In our clinic, we counsel adolescents about the need for regular and effective contraception while taking cyclophosphamide due to the possible negative effects on a developing fetus if there is an unplanned pregnancy. Recent evidence has shown that low-dose oral contraceptive pills are safe for women with lupus and are not associated with increased flares (19). Use of depo-provera injections for contraception, although highly effective, has been shown to have a negative impact on bone density if used over a long time (20). In adolescents with SLE, whose bone density is already impacted by chronic use of steroids, this side effect must be balanced with the need for contraceptive efficacy. The decision of which contraceptive is best should be an individual decision by the adolescent, rheumatologist, family doctor, and possibly a gynecologist.

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