Peterson L S, Nelson A M, Su W P, Mason T, O'Fallon W M, Gabriel S E
Department of Health Sciences Research, Mayo Clinic, Rochester, MN 55905, USA.
J Rheumatol. 1997 Jan;24(1):73-80.
To determine the incidence, prevalence, survival rates, clinical manifestations, and longterm outcome of patients with morphea (localized scleroderma) and its subtypes over a 33 year period in Olmsted County, Minnesota.
We used the unique data resources of the Rochester Epidemiology Project to review all Olmsted County medical records with any potential diagnosis consistent with morphea (including plaque, generalized, bullous, linear, and deep entities) from 1960 through 1993.
We screened 1030 medical records and identified 82 (59 female; 23 male) cases of morphea first diagnosed between 1960 and 1993. All cases were followed until death or migration from Olmsted County, a total of 754 person-years of observation. The annual age and sex adjusted incidence rate per 100,000 population was 2.7 (95% confidence interval 2.1, 3.3). The incidence rate increased significantly over the 33 years (p = 0.0037) on an average of 3.6% per year. The prevalence (estimated using cumulative incidence) at 80 years of age was about 2/1000. 50% of the patients had a cutaneous softening or evidence of disease resolution by 3.8 years' duration. The shortest active disease duration was found in the plaque group (50% resolution or skin softening by 2.7 years) compared to 5.5 years in the deep group. Arthralgias, synovitis, uveitis, and joint contractures were more frequent in the linear and deep categories. Although 9 patients (11%) developed some disease related disability over the followup period, this was common (44%) in the deep group. No case of morphea developed severe internal organ involvement and none progressed to systemic sclerosis. The survival rate was not significantly different from the general population (p = 0.409).
Morphea, and its subtypes, are more common than previously recognized, and can lead to important disability.
确定在明尼苏达州奥姆斯特德县33年期间硬斑病（局限性硬皮病）及其亚型患者的发病率、患病率、生存率、临床表现和长期转归。
我们利用罗切斯特流行病学项目的独特数据资源，回顾了1960年至1993年奥姆斯特德县所有有任何与硬斑病（包括斑块状、全身性、大疱性、线状和深部病变）一致的潜在诊断的医疗记录。
我们筛查了1030份医疗记录，确定了82例（59例女性；23例男性）于1960年至1993年首次诊断的硬斑病病例。所有病例均随访至死亡或从奥姆斯特德县迁出，共观察754人年。每10万人口中经年龄和性别调整的年发病率为2.7（95%置信区间2.1, 3.3）。在33年期间发病率显著上升（p = 0.0037），平均每年上升3.6%。80岁时的患病率（使用累积发病率估算）约为2/1000。50%的患者在病程3.8年时有皮肤软化或疾病缓解迹象。斑块状组疾病最短活动期（50%缓解或皮肤软化在2.7年时）较深部组的5.5年短。关节痛、滑膜炎、葡萄膜炎和关节挛缩在线状和深部类型中更常见。虽然9例患者（11%）在随访期间出现了一些与疾病相关的残疾，但在深部组中这一情况很常见（44%）。没有硬斑病病例发生严重的内脏受累，也没有进展为系统性硬化症。生存率与一般人群无显著差异（p = 0.409）。
硬斑病及其亚型比以前认识到的更常见，并且可导致严重残疾。
