Upon return, she is found to be in acute kidney injury, with peripheral edema, hypertension, and abnormal laboratory parameters as noted in Table 1. She admits that she has not been adherent to her drug regimen and states she felt “well” until just this past week. In the interim, she lost her supplemental drug coverage that paid for her MMF. Prednisone was reinstituted along with IV cyclophosphamide using the EuroLupus dosing regimen. She demonstrated a slow and consistent improvement, and laboratory parameters six months following reinstitution of therapy continued towards normalization. Current medications include hydroxychloroquine, a slow prednisone taper schedule, and mycophenolate sodium following re-induction. She has recently become sexually active, and states that she “sometimes” forgets her medications. She attends school “most days,” but is not happy with her appearance, and has difficulty concentrating and remembering what she is learning in school.
Management
There are several unique issues to consider in the management of cSLE. While the principles of drug therapy are similar to aSLE, the treatment of the child/adolescent requires greater involvement of the physician and other healthcare providers. In addition to the usual issues in caring for adults with SLE, the treating team must be mindful of normal expected growth and development, pubertal status, age and stage of adolescence, maturity, self-management capacity, and education and involvement of the family. The usual adolescent issues are magnified by the addition of a chronic disease, while fatigue, joint pain, and “lupus fog” make school and extracurricular participation difficult. Medication adherence is a significant challenge, and side effects have a significant impact on the ability and confidence of the adolescent to establish new friendships and intimate relationships that are crucial during this period. The “sex, drugs, and rock ‘n roll” phase of adolescence (i.e., periods of normal experimentation) is altered by the disease and its treatment, which can contribute to both abnormal socialization and mood disorders.
Medication choices in cSLE are derived or extrapolated from aSLE clinical trials, with modifications made based on observational data and the severity of organ involvement. Drug dosing is based on pharmacokinetic considerations including age, weight, and body surface area. Recent studies suggest more than 90% of patients with cSLE require prednisone at some point in their disease course, despite the fact that its side effects including weight gain, acne, cushingoid facies, striae, increased facial hair, and mood alterations may be devastating to the adolescent and child alike.4,10 Antimalarial drugs are prescribed almost universally to patients with cSLE. Immunosuppressants, including methotrexate, azathioprine, leflunomide, and MMF, are prescribed for patients with organ involvement and for those who are unable to successfully taper off prednisone. The Aspreva Lupus Management Study (ALMS) compared MMF versus cyclophosphamide for LN. It was the first clinical trial for LN to include cSLE patients over 12 years old. Examination of the study’s 24 cSLE subjects in the ALMS study suggested no difference in response rate between the drugs for induction of LN, but the study lacked adequate power for this subgroup analysis.11
