Celińska-Löwenhoff Magdalena, Pastuszczak Maciej, Pełka Karolina, Stec-Polak Magdalena, Wojas-Pelc Anna, Musiał Jacek
2 Department of Medicine, Jagiellonian University Medical College, Krakow, Poland.
Department of Dermatology, Jagiellonian University Medical College, Krakow, Poland.
Arch Med Sci. 2019 Jan 11;16(2):297-301. doi: 10.5114/aoms.2018.81129. eCollection 2020.
Mixed connective tissue disease (MCTD) is a chronic immune-mediated disorder defined by the combined presence of serum anti-RNP antibodies and distinct clinical features including progressive lung fibrosis. The aim of this study was to evaluate potential associations between lung fibrosis in MCTD and specific clinical and laboratory findings including results of nailfold capillaroscopy (NFC) examination.
Patients with MCTD who were admitted to the Departments of Allergy and Immunology or Dermatology at the University Hospital of Krakow (Poland) in 2015-2018 were identified based on comprehensive individual record review. Diagnosis of MCTD required fulfilment of at least one of the four widely accepted sets of diagnostic criteria. Clinical and laboratory data were collected, and statistical analysis was performed to identify potential predictors of interstitial lung disease (ILD).
Thirty patients (90% females) aged 22-81 years met the study inclusion criteria. The mean duration of symptoms associated with MCTD was 7.3 months. Photosensitivity and Raynaud's phenomenon were the most common clinical manifestations (90% and 70%, respectively). Hand oedema, sclerodactyly and the presence of giant capillaries in NFC correlated significantly with the risk of lung involvement. In multivariate analysis, the presence of enlarged loops in NFC (giant capillaries) was identified as an independent factor for ILD ( = 0.82, < 0.0000001).
The NFC examination should be considered in all patients with a diagnosis of MCTD. The presence of giant capillaries may be a promising marker for interstitial lung disease in these patients, especially among those with a short duration of disease (i.e. < 1 year).
混合性结缔组织病（MCTD）是一种慢性免疫介导性疾病，其定义为血清抗RNP抗体的联合存在以及包括进行性肺纤维化在内的独特临床特征。本研究的目的是评估MCTD中的肺纤维化与特定临床和实验室检查结果（包括甲襞毛细血管镜检查（NFC）结果）之间的潜在关联。
通过全面的个人病历审查，确定了2015年至2018年在波兰克拉科夫大学医院过敏与免疫学或皮肤科住院的MCTD患者。MCTD的诊断需要满足至少一套广泛接受的四项诊断标准中的一项。收集临床和实验室数据，并进行统计分析以确定间质性肺疾病（ILD）的潜在预测因素。
30名年龄在22至81岁之间的患者（90%为女性）符合研究纳入标准。与MCTD相关的症状平均持续时间为7.3个月。光敏性和雷诺现象是最常见的临床表现（分别为90%和70%）。手部水肿、指端硬化和NFC中出现巨大毛细血管与肺部受累风险显著相关。在多变量分析中，NFC中出现增大的襻（巨大毛细血管）被确定为ILD的独立因素（ = 0.82， < 0.0000001）。
所有诊断为MCTD的患者均应考虑进行NFC检查。巨大毛细血管的存在可能是这些患者间质性肺疾病的一个有前景的标志物，尤其是在病程较短（即<1年）的患者中。
