Uziel Y, Feldman B M, Krafchik B R, Yeung R S, Laxer R M
Division of Rheumatology and Pediatric Medicine, Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
J Pediatr. 2000 Jan;136(1):91-5. doi: 10.1016/s0022-3476(00)90056-8.
Localized scleroderma (LS) can cause permanent disability, and there is no universally accepted effective treatment. Methotrexate (MTX) has been shown to be effective in the treatment of systemic sclerosis.
To determine the efficacy and tolerability of MTX and corticosteroid therapy in patients with LS.
MTX, 0.3 to 0.6 mg/kg per week, was given to 10 patients (6 girls, 4 boys; mean age, 6.8 years; mean disease duration before starting treatment, 4 years) with active LS. In addition, pulse intravenous methylprednisolone, 30 mg/kg for 3 days monthly for 3 months, was given to 9 patients at the initiation of therapy.
One patient discontinued taking MTX after a month; the remaining 9 patients responded. The median time to response was 3 months (95% CI, 1.15-4.85). One responder discontinued taking MTX after a year because of leukopenia; the LS worsened within 2 months. In another patient LS flared up after 10 months and responded to an increased dose of MTX and intravenous methylprednisolone. At the last follow-up visit, all patients who continued to receive MTX therapy had inactive skin lesions.
Treatment with MTX and corticosteroids appears to be effective in the treatment of LS and is generally well tolerated. A placebo-controlled study is necessary to confirm the efficacy of MTX therapy in LS.
局限性硬皮病(LS)可导致永久性残疾,且尚无普遍认可的有效治疗方法。甲氨蝶呤(MTX)已被证明对系统性硬化症的治疗有效。
确定MTX和皮质类固醇疗法对LS患者的疗效和耐受性。
每周给予0.3至0.6mg/kg MTX治疗10例(6名女孩,4名男孩;平均年龄6.8岁;开始治疗前平均病程4年)活动性LS患者。此外,9例患者在治疗开始时每月静脉注射甲基强的松龙脉冲剂量30mg/kg,共3天,持续3个月。
1例患者在1个月后停止服用MTX;其余9例有反应。反应的中位时间为3个月(95%CI,1.15 - 4.85)。1例有反应者在1年后因白细胞减少而停止服用MTX;LS在2个月内恶化。另1例患者在10个月后LS复发,增加MTX剂量和静脉注射甲基强的松龙后有反应。在最后一次随访时,所有继续接受MTX治疗的患者皮肤病变均无活动。
MTX和皮质类固醇治疗似乎对LS有效,且总体耐受性良好。需要进行安慰剂对照研究以证实MTX疗法对LS的疗效。