2008
DOI: 10.1007/s10067-008-0973-2
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Treatment of refractory juvenile dermatomyositis with tacrolimus

Abstract: We report the clinical course of three patients with refractory juvenile dermatomyositis (JDM) who were treated with tacrolimus. All three children had extensive skin disease and severe muscle weakness and were corticosteroid dependent. All three patients showed impressive improvement of mainly the cutaneous lesions. Furthermore, overall disease activity decreased, all children became more physically active, and corticosteroid treatment could be tapered. However, none of the patients showed recovery of muscle … Show more

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Cited by 45 publications
(13 citation statements)
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“…In a retrospective trial of 50 children with JDM treated with mycophenolate mofetil, Disease Activity Score improved and prednisone dose was decreased after 12 months [46]. Systemic tacrolimus therapy was shown to improve refractory skin disease as well as overall disease activity in a case series of three children with JDM [47]. Finally, hydroxychloroquine has been reported to be effective in patients with an incomplete response to glucocorticoid therapy [48].…”
Section: Other Agentsmentioning
confidence: 98%
“…In a retrospective trial of 50 children with JDM treated with mycophenolate mofetil, Disease Activity Score improved and prednisone dose was decreased after 12 months [46]. Systemic tacrolimus therapy was shown to improve refractory skin disease as well as overall disease activity in a case series of three children with JDM [47]. Finally, hydroxychloroquine has been reported to be effective in patients with an incomplete response to glucocorticoid therapy [48].…”
Section: Other Agentsmentioning
confidence: 98%
“…Small case-series suggest the well-tolerated and effective role of tacrolimus in JDM [15]. The improvement in both skin and muscle disease activity and a steroid-sparing effect of both MMF and tacrolimus should be confirmed in larger prospective trials [14,15]. In extremely severe cases, resistant to multiple immunosuppressive therapies (alone or in combination), the autologous stem-cell transplant remains an option that has been successfully reported in two children who achieved remission [16].…”
Section: Therapy For Jdmmentioning
confidence: 90%
“…Only one retrospective study with 50 patients with JDM, supports the use of mycophenolate mofetil (MMF) as second-line treatment for patients with partial response to aggressive parenteral corticosteroids and MTX [14]. Small case-series suggest the well-tolerated and effective role of tacrolimus in JDM [15]. The improvement in both skin and muscle disease activity and a steroid-sparing effect of both MMF and tacrolimus should be confirmed in larger prospective trials [14,15].…”
Section: Therapy For Jdmmentioning
confidence: 94%
“…Similarly, tacrolimus has been reported to induce beneficial effects in children with refractory JDM, especially in those with severe skin involvement [51]. Intravenous cyclophosphamide pulse therapy was used in 12 JDM patients, with a significant improvement in muscle function, muscle strength, extramuscular disease activity score and cutaneous disease in 10/12 patients at 6-month follow-up.…”
Section: Difficult To Treat Patientsmentioning
confidence: 98%