Clinical features of patients with morphea and the pansclerotic subtype: a cross-sectional study from the morphea in adults and children cohort.
Clinical features of patients with morphea and the pansclerotic subtype: a cross-sectional study from the morphea in adults and children cohort.
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DOI：
10.3899/jrheum.130029
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发表时间：
2014-01
期刊：
影响因子：
--
通讯作者：
Jacobe HT
中科院分区：
文献类型：
--
作者：
Kim A;Marinkovich N;Vasquez R;Jacobe HT
全硬斑病是一种描述不多的硬斑病，关于患病率、人口统计学和临床特征的信息很少。这种亚型的分类标准各不相同，与其他形式的硬斑病如广泛全身性硬斑病和泛硬斑病的区别并不总是很清楚。本研究的目的是通过确定全硬斑病在成人和儿童硬斑病（MAC）队列中的患病率并描述其人口统计学和临床特征，以阐明全硬斑病的分类标准。使用改良的Laxer和Zulian分类系统确定符合全身性和全硬脑膜炎标准的患者。收集患者的基线人口统计学和临床特征，然后分析全硬斑病与泛发性硬斑病的特征。113例符合入选标准- 13例为泛发性硬斑病，100例为全身性硬斑病。胰腺炎患者多为男性（46.2% vs. 6%，p<0.0001）;诊断时间较短（平均差异为10.4个月[95% CI：0.8-19.9个月]，p=0.0332）;功能损害发生率较高（61.5% vs. 16%，p=0.0046）;深度受累率较高（61.5% vs. 17%，p=0.004）;平均mRSS更高（平均差异为10.8分[95% CI：5-16.6]，p=0.0018），LoSDI（平均差异28.3 [95% CI：9-47.6]，p=0.009）和PGA-D评分（平均差异25.1 [95% CI：0.3-50]，p=0.048）。我们的研究结果表明，人口统计学和临床特征足以定义泛甲状腺肿亚型，因为它们代表了一种独特的临床表型，具有更快速的进展和严重的过程，通常伴有残疾。泛血小板表型的特征的存在应提醒从业者显著发病的可能性和早期积极治疗的必要性。
Pansclerotic morphea is a poorly described form of morphea with little information on prevalence, demographics, and clinical features. Classification criteria for this subtype varies and the distinction from other forms of morphea such as extensive generalized morphea and pansclerotic morphea is not always clear. The purpose of this study was to clarify classification criteria for pansclerotic morphea by identifying its prevalence in the morphea in adults and children (MAC) cohort and describing its demographic and clinical features as compared with generalized morphea. Patients who met predefined criteria for generalized and pansclerotic morphea were identified using a modified Laxer and Zulian classification system. Baseline demographic and clinical features of the patients were compiled and then analyzed for traits characteristic of pansclerotic morphea versus those of generalized morphea. 113 met criteria for inclusion – 13 pansclerotic and 100 generalized morphea type. Pansclerotic patients were more frequently male (46.2% vs. 6%, p<0.0001); had a shorter time to diagnosis (mean difference of 10.4 months [95% CI: 0.8-19.9 months], p=0.0332); higher rates of functional impairment (61.5% vs. 16%, p=0.0046); higher rates of deep involvement (61.5% vs. 17%, p=0.004); and higher average mRSS (mean difference of 10.8 points [95% CI: 5-16.6], p=0.0018), LoSDI (mean difference 28.3 [95% CI: 9-47.6], p=0.009), and PGA-D scores (mean difference 25.1 [95% CI: 0.3-50], p=0.048). Our results suggest demographic and clinical features are sufficient to define the pansclerotic subtype as they represent a distinct clinical phenotype with a more rapidly progressive and severe course commonly accompanied by disability. Presence of features of the pansclerotic phenotype should alert practitioners to the possibility of significant morbidity and the need for early aggressive treatment.
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DOI：
10.3899/jrheum.081284
发表时间：
2009-12
期刊：
The Journal of rheumatology
影响因子：
--
作者：
Arkachaisri T;Vilaiyuk S;Li S;O'Neil KM;Pope E;Higgins GC;Punaro M;Rabinovich EC;Rosenkranz M;Kietz DA;Rosen P;Spalding SJ;Hennon TR;Torok KS;Cassidy E;Medsger TA Jr;Localized Scleroderma Clinical and Ultrasound Study Group
通讯作者：
Localized Scleroderma Clinical and Ultrasound Study Group
影响因子：
5.5
作者：
Arkachaisri, Thaschawee;Vilaiyuk, Soamarat;Medsger, Thomas A., Jr.
通讯作者：
Medsger, Thomas A., Jr.
影响因子：
1.8
作者：
Doede, T;Wollina, U;Bondartschuk, M
通讯作者：
Bondartschuk, M
影响因子：
13.8
作者：
Fett, Nicole;Werth, Victoria P.
通讯作者：
Werth, Victoria P.
影响因子：
--
作者：
ROOK, AH;FREUNDLICH, B;EDELSON, RL
通讯作者：
EDELSON, RL