Tang Keyun, Zhang Hanlin, Jin Hongzhong
Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China.
Front Med (Lausanne). 2021 Dec 2;8:783416. doi: 10.3389/fmed.2021.783416. eCollection 2021.
Clinical amyopathic dermatomyositis (CADM) represents a subtype of 5-20% of patients with dermatomyositis (DM), which can be categorized into amyopathic dermatomyositis (ADM) and hypomyopathic dermatomyositis (HDM). The characteristics of patients with CADM are still limited in English literature. To investigate clinical features, cutaneous findings, diagnostic accuracy, and treatment regimen of CADM patients. Sixty-four patients diagnosed with CADM at Peking Union Medical College Hospital by dermatologists were retrospectively analyzed. Data were recorded in the electronic database at each offline clinical consultation and directly extracted from medical records. 2017 EULAR/ACR criteria for idiopathic inflammatory myositis (IIM) classification was used to identify and classify patients with CADM. Published studies were searched to extract relevant data of CADM patients. This cohort included 38 ADM patients and 26 HDM patients. 2017 EULAR/ACR criteria classified 67.2% of patients with CADM into probable or definite DM. Antimalarials were given to a majority of CADM patients (72.6%, = 45). However, 68.8% (31 out of 45) required at least one aggressive agent combined with hydroxychloroquine due to insufficient response or side effects. The median of systemic treatments in HDM was significantly higher than ADM ( = 0.007). The number of ADM patients using antimalarials as monotherapy was significantly higher than that of HDM patients ( = 0.031), while the number of HDM patients receiving steroids combined with immunosuppressants was significantly higher ( = 0.025). The median of Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) score improvement was 11.5 and 10.5 for ADM and HDM after a median follow-up of 31.5 and 32.5 months, respectively. Six patients with normal muscle strength developed muscle weakness after a median of 10.5 months (IQR 9-13), and elevated inflammatory markers at initial visit might indicate their muscle weakness development. 32.8% of patients may be overlooked using the three skin variables of 2017 EULAR/ACR criteria. The response rate to single hydroxychloroquine in our cohort was 68.8%. Detailed treatment modalities were different among ADM and HDM. Long-term monitoring for the development of myositis in patients with CADM, especially those with elevated inflammatory markers at initial visit, may be warranted.
临床无肌病性皮肌炎（CADM）是皮肌炎（DM）患者中占比5%-20%的一个亚型，可分为无肌病性皮肌炎（ADM）和低肌病性皮肌炎（HDM）。英文文献中关于CADM患者的特征描述仍然有限。为了研究CADM患者的临床特征、皮肤表现、诊断准确性及治疗方案。对北京协和医院皮肤科诊断为CADM的64例患者进行回顾性分析。每次线下临床会诊时将数据记录在电子数据库中，并直接从病历中提取。采用2017年欧洲抗风湿病联盟/美国风湿病学会（EULAR/ACR）特发性炎性肌病（IIM）分类标准对CADM患者进行识别和分类。检索已发表的研究以提取CADM患者的相关数据。该队列包括38例ADM患者和26例HDM患者。2017年EULAR/ACR标准将67.2%的CADM患者分类为可能或确诊的DM。大多数CADM患者（72.6%，n = 45）接受了抗疟药治疗。然而，68.8%（45例中的31例）因疗效不佳或出现副作用而需要至少一种强效药物联合羟氯喹治疗。HDM患者全身治疗的中位数显著高于ADM患者（P = 0.007）。ADM患者使用抗疟药作为单一疗法的人数显著高于HDM患者（P = 0.031），而接受类固醇联合免疫抑制剂治疗的HDM患者人数显著更多（P = 0.025）。ADM和HDM患者在分别经过31.5个月和32.5个月的中位随访后，皮肤皮肌炎疾病面积和严重程度指数（CDASI）评分改善的中位数分别为11.5和10.5。6例肌力正常的患者在中位时间10.5个月（四分位间距9-13）后出现肌无力，初诊时炎症标志物升高可能提示其肌无力的发展。使用2017年EULAR/ACR标准的三个皮肤变量可能会遗漏32.8%的患者。我们队列中单一使用羟氯喹的有效率为68.8%。ADM和HDM的详细治疗方式有所不同。对于CADM患者，尤其是初诊时炎症标志物升高的患者，可能需要对肌炎的发展进行长期监测。
