Drug Therapy in Juvenile Dermatomyositis: Follow-Up Study

Abstract

A series of 33 patients with juvenile dermatomyositis was reviewed in terms of their prognosis in relation to their drug therapy. This retrospective study was intended to help clarify the use of various therapies in this rare, heterogeneous disease from our hospital's experience in the last 24 years. The results confirmed that oral corticosteroids should remain the undisputed first line of treatment. For more refractory, chronic patients, the results suggest that azathioprine should be the favored drug of first choice (in addition to corticosteroids). There may be a role for cyclosporine as a "rescue" treatment, but this needs to be further defined.

Long-term outcomes in juvenile dermatomyositis: How did we get here and where are we going?

“…Unfortunately, most of these reports continue to focus on survival, disease activity, and calcinosis. In 1998, Ng et al [23] reported on 33 children with JDM, followed-up for 2 to 18 years. All received corticosteroids, and 14 received at least one of cyclosporine, azathioprine, or methotrexate.…”

Therapy of polymyositis and dermatomyositis

“…Increase of liver enzymes is the main toxic effect in azathioprine-treated patients [9]. Azathioprine efficacy in PM/DM is mainly supported by a small number of case reports [9,[53][54][55]. In a small series, 12% of PM/ DM patients responded completely to azathioprine, 52% responded partially and 36% did not improve at all [56].…”

The treatment and prognosis of dermatomyositis: An updated review

“…65 However, patients with JDM treated with azathioprine have reduced corticosteroid doses, 26 and it has helped patients who failed other immunosuppressive therapies. 37 It is difficult to assess the effectiveness of azathioprine in DM. It is certainly an acceptable second-line therapy based on the current literature, although methotrexate may have a better side-effect profile.…”

“…Relapse rates, however, have varied from study to study with monophasic disease (patients with only one flare of myositis and no relapses) varying from 8% to 51.4%. 25,27,[33][34][35] In JDM, monophasic disease has ranged from 25% to 59.1%, 34,[36][37][38][39] and these patients use corticosteroids for shorter times, 32,36,37,[40][41][42] with one study finding corticosteroid treatment necessary for an average of only 14 months. 42 Another study 36 suggested that low-dose maintenance therapy does little to prevent relapse and may be harmful because of the side effects of corticosteroids.…”

“…28,37,[58][59][60] It has also had a corticosteroid-sparing effect. 59 Additionally, it can shorten the duration of corticosteroid therapy in JDM.…”