Cutaneous dermatomyositis disease course followed over time using the Cutaneous Dermatomyositis Disease Area and Severity Index.

Cutaneous dermatomyositis disease course followed over time using the Cutaneous Dermatomyositis Disease Area and Severity Index.
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DOI:
10.1016/j.jaad.2017.10.022
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发表时间:
2018-09
影响因子:
13.8
通讯作者:
Werth, Victoria P.
Werth, Victoria P.
中科院分区:
医学1区
文献类型:
--
作者:
Chansky, Peter B.;Olazagasti, Jeannette M.;Feng, Rui;Werth, Victoria P.

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有限的研究描述了皮肤皮肌炎(DM)的纵向过程。使用经验证的结局工具皮肤皮肌炎疾病面积和严重程度指数(CDASI)描述治疗的皮肤DM的病程。回顾性队列包括CDASI活动分项评分记录至少2年的DM患者。疾病进展分为改善、恶化或稳定,病程分为单相、多相或慢性。受试者分为轻度(n=16)和中重度(n=24)基线严重程度组。40例DM患者符合标准。与疾病恶化(n=7,17.5%)和疾病稳定(n =12,30.0%)相比,大多数患者的疾病活动性改善(n =21,52.5%)。大多数轻度疾病患者保持稳定(n=10,62.5%),而大多数中度至重度疾病患者改善(n=19,79.2%)。多相过程(n=33,82.5%)占主导地位,相比之下,单相(n=5,12.5%)和慢性(n=2,5%)的过程。平均发作/年与基线疾病活动无关。回顾性设计、潜在的转诊偏倚和分类标准的截止值是局限性。基线CDASI活动评分与皮肤DM的病程和进展的特定模式相关。
Limited studies describe the longitudinal course of cutaneous dermatomyositis (DM). To characterize disease course in treated cutaneous DM using the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), a validated outcome instrument. A retrospective cohort included patients with DM who had the CDASI activity subscore recorded for at least 2 years. Disease progression was classified into improved, worsened, or stable and disease course was classified into monophasic, polyphasic, or chronic. Subjects were divided into mild (n=16) and moderate-severe (n=24) baseline severity groups. Forty DM patients met criteria. The majority demonstrated improvement in disease activity (n=21, 52.5%) compared to worsening (n=7, 17.5%) and stable (n=12, 30.0%) disease. Most patients with mild disease remained stable (n=10, 62.5%) whereas most with moderate to severe disease improved (n=19, 79.2%). A polyphasic course (n=33, 82.5%) predominated, compared to monophasic (n=5, 12.5%) and chronic (n=2, 5%) courses. Average flares/year was independent of baseline disease activity. Retrospective design, potential referral bias, and cutoff-values in classification criteria are limitations. Baseline CDASI activity score is associated with particular patterns of disease course and progression in cutaneous DM.
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