儿童局限性硬皮病发病队列的临床特征。
Clinical features of childhood localized scleroderma in an incidence cohort.
机构信息
Arthritis Research UK Epidemiology Unit, University of Manchester, Stopford Building, Oxford Road, Manchester M13 9PT, UK.
出版信息
Rheumatology (Oxford). 2011 Oct;50(10):1865-8. doi: 10.1093/rheumatology/ker142. Epub 2011 Jul 5.
OBJECTIVES
Our aim was to describe clinical features and pattern of care in children with localized scleroderma presenting to secondary care during a 25-month incidence study.
METHODS
Eighty-seven patients were identified, and clinical features, serum autoantibodies, current treatment and outcome at 12 months were documented.
RESULTS
Fifty-eight (67%) had linear scleroderma, 25 (29%) non-linear morphoea and 4 (4%) a mixed pattern. Of the 58 patients with linear scleroderma, 29 (50%) presented with lesions of the trunk and/or limbs only, 26 (45%) with face-head localization only and 3 (5%) with both. Thirteen (15%) had extracutaneous features and 16 (43%) out of 37 were ANA positive. At 12 months, 59% were on MTX. At 12 months, 51 (65%) were improved/resolved, 14 (18%) were unchanged and 13 (17%) had deteriorated.
CONCLUSION
Key findings included the high prevalence of face-head involvement in those with linear disease, and the high prevalence of extracutaneous disease and of ANA positivity. After 12 months, most patients improved according to clinician's opinion.
目的
本研究旨在描述在为期 25 个月的发病研究期间，于二级医疗机构就诊的局限性硬皮病患儿的临床特征和治疗模式。
方法
共确定了 87 例患者，记录了他们的临床特征、血清自身抗体、当前治疗情况以及 12 个月时的转归。
结果
58 例（67%）为线状硬皮病，25 例（29%）为非线状硬肿病，4 例（4%）为混合模式。在 58 例线状硬皮病患者中，29 例（50%）仅表现为躯干和/或四肢皮损，26 例（45%）仅表现为头面部皮损，3 例（5%）同时存在上述两种皮损。13 例（15%）有皮肤外表现，37 例中有 16 例（43%）ANA 阳性。12 个月时，59%的患者接受 MTX 治疗。12 个月时，51 例（65%）患者改善/缓解，14 例（18%）患者病情无变化，13 例（17%）患者病情恶化。
结论
主要发现包括线状皮损患者中头面部受累的高发生率，以及皮肤外疾病和 ANA 阳性的高发生率。12 个月后，大多数患者根据临床医生的意见得到改善。
Zotero 插件