Bogojevic Milan, Markovic Vlaisavljevic Milica, Medjedovic Rifat, Strujic Elvira, Pravilovic Lutovac Dragana, Pavlov-Dolijanovic Slavica
Department of Rheumatology, Clinical Centre of Montenegro, 81000 Podgorica, Montenegro.
Faculty of Medicine, Institute of Rheumatology, University of Belgrade, 11000 Belgrade, Serbia.
J Clin Med. 2024 Sep 19;13(18):5550. doi: 10.3390/jcm13185550.
Idiopathic inflammatory myopathies (IIMs) are rare autoimmune disorders characterized by progressive proximal muscle weakness and varying extra-muscular manifestations. The latest 2017 EULAR/ACR criteria classify them into subgroups. This study aims to evaluate the role of nailfold capillaroscopy (NFC) as a diagnostic and prognostic tool in IIMs by comparing capillaroscopic patterns across different IIM subtypes. We conducted an observational, cross-sectional study at the Institute of Rheumatology in Belgrade, analyzing 90 patients diagnosed with IIMs per the 2017 EULAR/ACR criteria. Patients were categorized into dermatomyositis (DM) (n = 37), polymyositis (PM) (n = 35), amyopathic dermatomyositis (ADM) (n = 13), and juvenile dermatomyositis (JDM) (n = 5). A control group of 35 patients with primary Raynaud's phenomenon was also included. NFC findings, clinical manifestations, and laboratory data were compared across the groups. In DM, 81.9% exhibited a scleroderma capillaroscopic pattern, which was also present in 76.9% of ADM patients. In PM, the most common pattern was nonspecific changes (48.6%). JDM patients showed a high prevalence of scleroderma changes (n = 4 (80%)). Scleroderma patterns correlated with Gottron's papules, heliotrope rash, periungual erythema, Raynaud's phenomenon, and interstitial lung disease (ILD). No significant differences were found in laboratory parameters across capillaroscopic groups, except for a higher prevalence of anti-Jo1 antibodies in patients with nonspecific capillaroscopic changes. NFC is a valuable tool for differentiating IIM subtypes and correlating clinical manifestations with specific capillaroscopic patterns. The high prevalence of scleroderma changes in DM and ADM suggests their potential as a diagnostic and prognostic marker in IIMs. Further research with larger cohorts is warranted to validate these findings.
特发性炎性肌病(IIMs)是罕见的自身免疫性疾病,其特征为进行性近端肌无力和各种肌肉外表现。2017年欧洲抗风湿病联盟(EULAR)/美国风湿病学会(ACR)的最新标准将其分为不同亚组。本研究旨在通过比较不同IIM亚型的甲襞毛细血管镜检查(NFC)模式,评估NFC作为IIM诊断和预后工具的作用。我们在贝尔格莱德风湿病研究所进行了一项观察性横断面研究,分析了90例根据2017年EULAR/ACR标准诊断为IIMs的患者。患者被分为皮肌炎(DM)(n = 37)、多发性肌炎(PM)(n = 35)、无肌病性皮肌炎(ADM)(n = 13)和青少年皮肌炎(JDM)(n = 5)。还纳入了35例原发性雷诺现象患者作为对照组。对各组的NFC检查结果、临床表现和实验室数据进行了比较。在DM中,81.9%表现出硬皮病毛细血管镜模式,ADM患者中这一比例为76.9%。在PM中,最常见的模式是非特异性改变(48.6%)。JDM患者硬皮病改变的患病率很高(n = 4(80%))。硬皮病模式与Gottron丘疹、向阳疹、甲周红斑、雷诺现象和间质性肺病(ILD)相关。除了非特异性毛细血管镜改变的患者中抗Jo1抗体的患病率较高外