Nagy Alexandra, Palmer Erik, Polivka Lorinc, Eszes Noemi, Vincze Krisztina, Barczi Eniko, Bohacs Aniko, Tarnoki Adam Domonkos, Tarnoki David Laszlo, Nagy György, Kiss Emese, Maurovich-Horvat Pal, Müller Veronika
Department of Pulmonology, Semmelweis University, 1083 Budapest, Hungary.
Medical Imaging Centre, Semmelweis University, 1082 Budapest, Hungary.
Biomedicines. 2022 Feb 13;10(2):434. doi: 10.3390/biomedicines10020434.
(1) Background: Systemic sclerosis (SSc) is frequently associated with interstitial lung diseases (ILDs). The progressive form of SSc-ILD often limits patient survival. The aim of our study is to evaluate the clinical characteristics and predictors of lung function changes in SSc-ILD patients treated in a real-world setting. (2) Methods: All SSc-ILD cases previously confirmed by rheumatologists and a multidisciplinary ILD team between January 2017 and June 2019 were included ( = 54). The detailed medical history, clinical parameters and HRCT were analyzed. The longitudinal follow-up for pulmonary symptoms, functional parameters and treatment were performed for at least 2 years in no treatment, immunosuppression and biological treatment subgroups. (3) Results: In SSc-ILD patients (age 58.7 ± 13.3 years, 87.0% women), the main symptoms included dyspnea, cough, crackles and the Raynaud's phenomenon. The functional decline was most prominent in untreated patients, and a normal body mass index (BMI < 25 kg/m) was associated with a significant risk of deterioration. The majority of patients improved or were stable during follow-up. The progressive fibrosing-ILD criteria were met by 15 patients, the highest proportion being in the untreated subgroup. (4) Conclusions: SSc-ILD patients who are overweight are at a lower risk of the functional decline and progressive phenotype especially affecting untreated patients. The close monitoring of lung involvement and a regular BMI measurement are advised and early treatment interventions are encouraged.
(1) 背景:系统性硬化症(SSc)常与间质性肺疾病(ILDs)相关。SSc-ILD的进展型常限制患者生存。我们研究的目的是评估在真实世界环境中接受治疗的SSc-ILD患者的临床特征及肺功能变化的预测因素。(2) 方法:纳入2017年1月至2019年6月期间经风湿病学家和多学科ILD团队先前确诊的所有SSc-ILD病例(n = 54)。分析详细病史、临床参数和高分辨率CT(HRCT)。对未治疗、免疫抑制和生物治疗亚组的患者进行至少2年的肺部症状、功能参数及治疗的纵向随访。(3) 结果:在SSc-ILD患者中(年龄58.7±13.3岁,87.0%为女性),主要症状包括呼吸困难、咳嗽、啰音和雷诺现象。未治疗患者的功能下降最为显著,正常体重指数(BMI<25kg/m²)与恶化的显著风险相关。大多数患者在随访期间病情改善或稳定。15例患者符合进行性纤维化ILD标准,其中未治疗亚组比例最高。(4) 结论:超重的SSc-ILD患者功能下降和进展型表型的风险较低,尤其在未治疗患者中。建议密切监测肺部受累情况并定期测量BMI,鼓励早期进行治疗干预。