Riley, P; Maillard, SM; Wedderburn, LR; Woo, P; Murray, KJ; Pilkington, CA; (2004) Intravenous cyclophosphamide pulse therapy in juvenile dermatomyositis. A review of efficacy and safety. Rheumatology (Oxford) , 43 (4) 491 - 496.
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Objectives. To assess the efficacy and safety of intravenous cyclophosphamide (CYP) used in severe and refractory juvenile dermatomyositis (JDM). Methods. Retrospective case note review of the outcome of 12 patients. RESULTS: Assessment at 6 months of therapy in 10 of the 12 patients showed a significant improvement in muscle function as assessed by the Childhood Myositis Assessment Scale (CMAS) (P = 0.012), muscle strength (P = 0.008), global extramuscular disease score (P = 0.008), skin disease severity (P = 0.015) and lactate dehydrogenase (P = 0.028). There were reductions in creatine kinase, alanine aminotransferase, prednisolone dose and ESR, but these did not reach statistical significance. Clinical improvement was maintained after CYP until the most recent follow-up (between 6 months and 7 yr) and no severe side-effects were seen. Reversible complications included lymphopenia, herpes zoster infections and alopecia. The median cumulative dose was 4.6 g/m(2) (range 3-9 g/m(2)). The available evidence suggests that, at the doses required, risks of malignancy, infertility and gonadal failure are low. Two patients with severe treatment-resistant disease died after one dose of CYP, both of whom were ventilated prior to commencement of CYP and were thought to have died as a result of their severe disease process, and too early for clinical benefit to be obtained from the drug. CONCLUSIONS: In this cohort of children with severe and refractory JDM, CYP appeared to have provided major clinical benefit with no evidence of serious toxicity in the short term
|Title:||Intravenous cyclophosphamide pulse therapy in juvenile dermatomyositis. A review of efficacy and safety|
|Additional information:||DA - 20040316 IS - 1462-0324 LA - eng PT - Journal Article SB - AIM SB - IM|
|Keywords:||complications, Cyclophosphamide, Dermatomyositis, Infection, methods, Myositis, Prednisolone, Skin, therapy|
|UCL classification:||UCL > School of Life and Medical Sciences > Faculty of Medical Sciences > Infection and Immunity (Division of)|
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