皮肌炎中的钙化症。

IF 5.2 2区 医学 Q1 RHEUMATOLOGY
Srijana Davuluri, Lorinda Chung, Christian Lood
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引用次数: 0

摘要

综述目的提供有关皮肌炎钙化症发病机制和治疗方法的最新文献:早期诊断和控制整体疾病活动是预防幼年皮肌炎钙化的基石。观察性队列研究显示,长期炎症状态和血管功能障碍特征(如数字溃疡和甲沟毛细血管异常)与钙化有关。中性粒细胞活化和线粒体功能障碍最近成为参与钙化病发病机制的潜在机制途径。摘要:皮肌炎中的钙化症是皮肤和其他组织中不溶性钙化合物的沉积。多达 75% 的幼年皮肌炎患者和多达 20% 的成年皮肌炎患者都患有钙化症。虽然它会导致患者严重发病,但我们对其发病机制尚不完全了解。手术切除虽然是姑息性治疗,但仍是治疗的主要手段,应提供给患者。所有可用的治疗方案都只有很低水平的证据。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Calcinosis in dermatomyositis.

Purpose of review: To provide the most recent literature on our understanding behind the pathogenesis and the treatment of calcinosis in dermatomyositis.

Recent findings: Early diagnosis and controlling the overall disease activity are cornerstones to prevent calcinosis in juvenile dermatomyositis. Observational cohort studies showed that prolonged state of inflammation and features of vascular dysfunction like digital ulcers and abnormal nailfold capillaries are associated with calcinosis. Neutrophil activation and mitochondrial dysfunction have recently emerged as potential mechanistic pathways involved in calcinosis pathogenesis. Few recent case series have alluded to the efficacy of topical and intralesional sodium thiosulfate, while JAK inhibitors appear to be newer promising therapy in juvenile dermatomyositis.

Summary: Calcinosis in dermatomyositis consists of deposition of insoluble calcium compounds in the skin and other tissues. It is prevalent in up to 75% of patients with juvenile dermatomyositis and up to 20% in adult dermatomyositis. While it leads to significant patient morbidity, we do not yet understand the pathogenesis in its entirety. Surgical excision although palliative is the mainstay of treatment and should be offered to patients. All available treatment options are only based on very low level of evidence.

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来源期刊
Current opinion in rheumatology
Current opinion in rheumatology 医学-风湿病学
CiteScore
9.70
自引率
2.00%
发文量
89
审稿时长
6-12 weeks
期刊介绍: A high impact review journal which boasts an international readership, Current Opinion in Rheumatology offers a broad-based perspective on the most recent and exciting developments within the field of rheumatology. Published bimonthly, each issue features insightful editorials and high quality invited reviews covering two or three key disciplines which include vasculitis syndromes, medical physiology and rheumatic diseases, crystal deposition diseases and rheumatoid arthritis. Each discipline introduces world renowned guest editors to ensure the journal is at the forefront of knowledge development and delivers balanced, expert assessments of advances from the previous year.
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