Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal.
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa, Lisbon, Portugal.
ARP Rheumatol. 2024 Jul-Sep;3(3):174-181. doi: 10.63032/BAYU2491.
We aimed to identify clinical and serological predictors of myositis in mixed connective tissue disease (MCTD).
We performed a nationwide, retrospective, multicentre study including adult-onset MCTD patients fulfilling at least one of the following diagnostic criteria: Sharp's, Kasukawa, Alarcón-Segovia, or Kahn's. Univariate analysis was performed using Chi-square, Fisher exact, Student's t or Mann-Whitney U tests, as appropriate. Multivariate analysis was performed using binary logistic regression.
Ninety-eight patients were included. Myositis was observed in 43.9% of patients, of whom 60.5% had myositis at disease onset. Proximal muscle weakness was described in 30 patients with muscle involvement (70%). Gastrointestinal involvement was identified in 28% and respiratory involvement in 29% of myositis patients. In the same subgroup of patients, 41.7% had a myopathic pattern on electromyography, and 47.1% had histological myositis features in the muscle biopsy. Fever (OR=6.96, p=0.022) was an independent predictor of myositis, regardless of sex, age at diagnosis, ancestry, and respiratory involvement. African ancestry (OR=8.39, p=0.019), leukopenia at the disease onset (OR 6.24, p=0.021), and younger age at diagnosis (OR=1.07/year, p=0.035) were identified as independent predictors of myositis at disease onset, regardless of sex and scleroderma pattern in capillaroscopy.
Myositis is a common manifestation of MCTD, even at the disease onset. African ancestry, leukopenia at the disease onset, younger age at diagnosis, and fever should prompt a thorough evaluation for myositis.
我们旨在确定混合性结缔组织病(MCTD)中肌炎的临床和血清学预测因子。
我们进行了一项全国性的回顾性多中心研究,纳入了符合以下至少一项诊断标准的成年发病的 MCTD 患者:Sharp、Kasukawa、Alarcón-Segovia 或 Kahn。使用卡方检验、Fisher 精确检验、Student t 检验或 Mann-Whitney U 检验进行单变量分析,视情况而定。使用二元逻辑回归进行多变量分析。
共纳入 98 例患者。43.9%的患者出现肌炎,其中 60.5%的患者在疾病发病时出现肌炎。30 例有肌肉受累的患者出现近端肌无力(70%)。28%的肌炎患者有胃肠道受累,29%的患者有呼吸系统受累。在同一亚组患者中,41.7%的肌电图显示肌病模式,47.1%的肌肉活检显示组织学肌炎特征。发热(OR=6.96,p=0.022)是肌炎的独立预测因子,与性别、诊断时年龄、祖源和呼吸系统受累无关。非裔祖源(OR=8.39,p=0.019)、发病时白细胞减少(OR=6.24,p=0.021)和诊断时年龄较小(OR=1.07/年,p=0.035)是发病时肌炎的独立预测因子,与性别和毛细血管镜下硬皮病模式无关。
肌炎是 MCTD 的常见表现,甚至在疾病发病时也是如此。非裔祖源、发病时白细胞减少、诊断时年龄较小和发热应促使对肌炎进行彻底评估。