This study examines the differences which may distinguish systemic lupus erythematosus (SLE) presenting in adult life or childhood. A common database was established, with analysis of clinical, serological and outcome features of a cohort of patients with SLE, with disease diagnosed before the age of 16 (n = 39) or after the age of 16 (n = 165). Disease onset was generally more severe in the childhood-onset patients. Cardiopulmonary disease was more common in the older-onset group, but major haematological manifestations were more frequent in the childhood-onset group. Serologically, anti-DNA, anti-Sm and anti-RNP antibodies and a low C3 were all found more frequently in the younger patients. Twice as many adult-onset cases had died at the time of the last follow-up (10 vs 5%), but this group had been followed for a longer period (average 7.5 yr, S.D. 3.9 for adults vs average 4.8 yr, S.D. 3.2 for children). However, the younger patients were twice as likely (82 vs 40%) to require high-dose prednisone, although the requirement for immunosuppressive agents was similar in the two groups. Clinicians should anticipate that children with SLE have a more severe disease onset than adults in general.

Approximately 15% of patients with systemic lupus erythematosus (SLE) will have the onset of their disease in childhood or adolescence. Due to the broad range of possible clinical features of SLE, the diagnosis may be difficult to make in a general pediatric or community setting. The common symptoms of SLE in children and adolescents include fever, fatigue, weight loss, arthritis, rash and renal disease. SLE is more common in non-Caucasian ethnic groups and should be considered in the differential diagnosis of a multisystem disease in these patients. In this article, the classification criteria for SLE are discussed, and an approach to making an accurate and timely diagnosis of this disease is considered.

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Objective. To review the clinical course of 16 children with recalcitrant dermatomyositis (DM), who were treated with oral methotrexate (MTX) in addition to prednisone.Methods. Sixteen patients with recalcitrant DM who were treated with MTX in addition to prednisone were followed between 1984 and 1990. The patients' clinical responses to treatment, including alterations in muscle strength and muscle enzyme levels, changes in prednisone dosage, and development of toxicity or complications were reviewed retrospectively.Results. All 12 patients who received MTX for at least 8 months regained normal muscle strength. In 11 of the 12, the prednisone dosage could eventually be tapered to 5 5 mg/day. Complications during MTX treatment required discontinuation of MTX in 5 patients, and were unrelated to the cumulative dose of the drug. Active disease recurred in 5 patients in whom MTX had been discontinued after apparent clinical remission had been achieved.Conclusion. MTX, in combination with prednisone, is a useful adjunct in the treatment of recalcitrant childhood DM. However, recurrence of disease after withdrawal of MTX suggests that the drug may have a suppressive, rather than a remittive, effect. Most children with dermatomyositis (DM) can be treated successfully with corticosteroids ( 1-3). However, some patients relapse after initial improvement, have an incomplete response, or develop untoward side effects. In these patients, a variety of immunosuppressive treatments, including methotrexate (MTX), azathioprine, cyclophosphamide, hydroxychloroquine, and plasmapheresis, have been used as adjuncts to corticosteroid therapy (4-9). Although no controlled trials of these agents in either childhood or adult DM have been reported, there are several reports of retrospectively analyzed patient series in which the efficacy of MTX in the treatment of adults with DM has been investigated (10,ll). In addition, several case reports suggest that MTX may be useful in the treatment of childhood DM. In this investigation, we evaluated the clinical courses of 16 children with recalcitrant DM treated with MTX in addition to prednisone, in order to examine the efficacy and safety of this treatment regimen . PATIENTS AND METHODSThirty-four children with definite childhood DM were followed up prospectively by the same observers, in the pediatric rheumatology clinic at the Floating Hospital for Infants and Children, between 1984 and1990. Sixteen of these patients had recalcitrant DM and were treated with MTX in addition to prednisone. These 16 children were the subjects of the present investigation.Data evaluated included sex, age at onset of DM, disease duration (pre-prednisone and pre-MTX), muscle strength (at presentation to the Floating Hospital and at initiation of MTX), clinical responses to treatment including changes in prednisone dosage and development of toxicity or complications, and duration of followup. Classification of the clinical course of each patient prior to the institution of