Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, Padova, Italy.
Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, Padova, Italy.
RMD Open. 2023 Aug;9(3). doi: 10.1136/rmdopen-2023-003121.
Patients with connective tissue diseases can develop interstitial lung disease (ILD), leading to a progressive fibrosing ILD (PF-ILD) phenotype in some cases. We aimed to investigate the occurrence of PF-ILD in idiopathic inflammatory myopathies (IIMs), and factors potentially predicting this phenotype. Secondary aims were to assess the radiological pattern and factors associated with IIMs-ILD.
Patients with IIMs from our multicentric prospective cohort were retrospectively evaluated. Data were recorded at IIMs and ILD diagnosis, and during follow-up. Patients with ILD were classified according to the predominant high-resolution CT (HRCT) pattern: non-specific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP) and organising pneumonia (OP). PF-ILD was defined according to the 2022 American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS) and Latin American Thoracic Society (ALAT) guidelines. Univariate and multivariate analyses were performed to identify factors associated to ILD and to PF-ILD.
Of 253 patients with IIMs, 125 (49%) had ILD: 99 (78%) at IIMs diagnosis and 26 (22%) during follow-up (21/26 within 5 years). Multivariate analysis identified anti-Jo-1, anti-MDA5, anti-Ro52, high score on manual muscle test, mechanic's hands and Raynaud's phenomenon as independently associated with ILD. The predominant HRCT pattern was NSIP (50% of patients), followed by UIP (28%) and OP (22%). At 1-year follow-up, PF-ILD occurred in 18% of IIMs-ILD. PF-ILD was predicted by anti-MDA5, heliotropic rash, xerostomia and xerophthalmia at univariate but not at multivariate analysis.
Patients with IIM should be carefully screened for ILD at IIMs diagnosis and yearly during follow-up. All patients with IIMs-ILD should be carefully monitored to capture ILD progression since a consistent proportion of them are expected to develop PF-ILD.
结缔组织疾病患者可发生间质性肺疾病（ILD），某些情况下导致进行性纤维化ILD（PF-ILD）表型。本研究旨在探讨特发性炎性肌病（IIM）中 PF-ILD 的发生情况，以及可能预测该表型的因素。次要目的是评估与 IIM-ILD 相关的影像学模式和因素。
对来自我们多中心前瞻性队列的 IIM 患者进行回顾性评估。在 IIM 和ILD 诊断时以及随访期间记录数据。根据主要高分辨率 CT（HRCT）模式对ILD 患者进行分类：非特异性间质性肺炎（NSIP）、寻常型间质性肺炎（UIP）和机化性肺炎（OP）。根据 2022 年美国胸科学会（ATS）、欧洲呼吸学会（ERS）、日本呼吸学会（JRS）和拉丁美洲胸科学会（ALAT）指南，将 PF-ILD 定义为 UIP 以外的所有 ILD。采用单变量和多变量分析来识别与ILD 和 PF-ILD 相关的因素。
在 253 例 IIM 患者中，125 例（49%）有ILD：99 例（78%）在 IIM 诊断时，26 例（22%）在随访时（26 例中有 21 例在 5 年内）。多变量分析确定抗-Jo-1、抗-MDA5、抗 Ro52、手动肌肉测试评分高、技工手和雷诺现象与ILD 独立相关。主要 HRCT 模式为 NSIP（50%的患者），其次是 UIP（28%）和 OP（22%）。在 1 年随访时，18%的 IIM-ILD 患者发生 PF-ILD。PF-ILD 在单变量分析中由抗-MDA5、向心性皮疹、口干和眼干预测，但在多变量分析中未预测。
在 IIM 诊断时和随访期间应每年仔细筛查 IIM 患者的ILD。所有 IIM-ILD 患者都应密切监测以捕捉ILD 进展，因为预计其中相当一部分患者会发展为 PF-ILD。
