背景:皮肌炎(DM)是一组自身免疫性特发性炎症性肌病,其特征是典型的皮肤体征和肌肉受累症状。这些疾病可能与副肿瘤综合征中的癌症有关,钙质沉着,间质性肺病,其他自身免疫性结缔组织疾病(重叠综合征),和雷诺现象。
方法:收集了43例DM患者的临床和毛细管镜检查数据。诊断基于Bohan-Peter和欧洲抗风湿病联盟/美国风湿病学会(EULAR/ACR)分类标准。此外,所有患者均进行甲褶毛细血管镜检查。
结果:在我们的队列中,8名患者出现重叠综合征,6人患有副肿瘤综合征,八人患有间质性肺病,九人患有钙质沉着症,其中两人也有癌症病理。74%的患者报告了雷诺现象。在甲褶毛细管镜检查时,84%的患者表现出巨大的毛细血管,81%分支毛细血管,两者都有70%。后者,特别是巨大的分支毛细血管,可以认为是DM特有的。突出的乳头状下静脉丛的检测与肺部受累有关。相比之下,毛细血管周围间隙的改变与DM的严重程度和预后相关.
结论:我们的结果强调了甲褶毛细血管镜检查在DM的诊断和预后中的有用性。根据结果和文献数据,糖尿病诊断标准应包括特定的甲皱毛细血管镜检查特征.
BACKGROUND: Dermatomyositis (DM) is a group of autoimmune idiopathic inflammatory myopathies characterized by typical cutaneous signs and symptoms of muscle involvement. The diseases can be associated with cancer in the paraneoplastic syndrome, calcinosis, interstitial lung disease, other autoimmune connective tissue diseases (in overlap syndrome), and Raynaud\'s phenomenon.
METHODS: Clinical and capillaroscopic data were gathered from 43 patients with DM. The diagnosis was based on the Bohan‒Peter and European League against Rheumatism / American College of Rheumatology (EULAR/ACR) classification criteria. In addition, nailfold capillaroscopy was performed in all patients.
RESULTS: In our cohort, eight patients had overlap syndrome, six had paraneoplastic syndrome, eight presented with interstitial lung disease, and nine had calcinosis, two of whom also had a cancerous pathology. Raynaud\'s phenomenon was reported in 74% of patients. Upon nailfold capillaroscopy, 84% of patients presented giant capillaries, 81% ramified capillaries, and 70% both. The latter, notably giant ramified capillaries, could be considered specific for DM. The detection of prominent subpapillary venous plexuses was associated with pulmonary involvement. In contrast, alterations of the pericapillary spaces were associated with the severity and prognosis of DM.
CONCLUSIONS: Our results underline the usefulness of nailfold capillaroscopy in the diagnosis and prognosis of DM. Based on the results and literature data, specific nailfold capillaroscopy features should be included in DM diagnostic criteria.