肌炎相关性间质性肺病的诊断：肌炎自身抗体线免疫分析的效用。

Diagnosis of myositis-associated interstitial lung disease: Utility of the myositis autoantibody line immunoassay.

机构信息

Department of Respiratory, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Central Clinical School, University of Sydney Faculty of Medicine and Health, Sydney, NSW, Australia; National Health and Medical Research Council (NHMRC) Centre of Research Excellence in Pulmonary Fibrosis, Australia.

Central Clinical School, University of Sydney Faculty of Medicine and Health, Sydney, NSW, Australia; National Health and Medical Research Council (NHMRC) Centre of Research Excellence in Pulmonary Fibrosis, Australia; Department of Rheumatology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.

出版信息

Respir Med. 2021 Oct;187:106581. doi: 10.1016/j.rmed.2021.106581. Epub 2021 Aug 21.

DOI:10.1016/j.rmed.2021.106581

PMID:34454312

Abstract

OBJECTIVES

The detection of myositis autoantibodies (MA) in patients with interstitial lung disease (ILD) has major implications for diagnosis and management, especially amyopathic and forme frustes of idiopathic inflammatory myositis-associated ILD (IIM-ILD). Use of the MA line immunoblot assay (MA-LIA) in non-rheumatological cohorts remains unvalidated. We assessed the diagnostic performance of the MA-LIA and explored combined models with clinical variables to improve identification of patients with IIM-ILD.

METHODS

Consecutive patients referred to a specialist ILD clinic, with ILD-diagnosis confirmed at multidisciplinary meeting, and MA-LIA performed within six months of baseline were included. Pre-specified MA-LIA thresholds were evaluated for IIM-ILD diagnosis.

RESULTS

A total 247 ILD patients were included (IIM-ILD n = 12, non-IIM connective tissue disease-associated ILD [CTD-ILD] n = 52, idiopathic interstitial pneumonia [IIP] n = 115, other-ILD n = 68). Mean age was 64.8 years, with 45.3% female, mean FVC 75.5% and DLCO 59.2% predicted. MA were present in 13.8% overall and 83.3% of IIM-ILD patients. The most common MA in IIM-ILD and non-IIM ILD patients were anti-Jo-1 (prevalence 40%) and anti-PMScl (29.2%) autoantibodies respectively. The pre-specified low-positive threshold (>10 signal intensity) had the highest discriminative capacity for IIM-ILD (AUC 0.86). Combining MA-LIA with age, gender, clinical CTD-manifestations and an overlap non-specific interstitial pneumonia/organising pneumonia pattern on HRCT improved discrimination for IIM-ILD (AUC 0.96).

CONCLUSION

The MA-LIA is useful to support a diagnosis of IIM-ILD as a complement to multi-disciplinary ILD assessment. Clinical interpretation is optimised by consideration of the strength of the MA-LIA result together with clinical and radiological features of IIM-ILD.

摘要

目的

肌炎自身抗体（MA）在间质性肺疾病（ILD）患者中的检测对诊断和治疗具有重要意义，尤其是在特发性炎症性肌病相关ILD（IIM-ILD）的无肌病型和顿挫型中。在非风湿病患者中使用 MA 线免疫印迹检测（MA-LIA）尚未得到验证。我们评估了 MA-LIA 的诊断性能，并探讨了与临床变量相结合的综合模型，以提高对 IIM-ILD 患者的识别能力。

方法

纳入连续就诊于ILD 专科门诊、多学科会议确诊ILD、且在基线时进行了 MA-LIA 检测的患者。评估了预定义的 MA-LIA 阈值对 IIM-ILD 的诊断价值。

结果

共纳入 247 例ILD 患者（IIM-ILD 患者 n=12，非特发性炎症性肌病相关结缔组织病ILD [CTD-ILD]患者 n=52，特发性间质性肺炎 [IIP]患者 n=115，其他ILD 患者 n=68）。患者平均年龄为 64.8 岁，女性占 45.3%，平均 FVC 为 75.5%，DLCO 为 59.2%。总体 MA 阳性率为 13.8%，IIM-ILD 患者的阳性率为 83.3%。在 IIM-ILD 和非 IIM-ILD 患者中最常见的 MA 分别为抗 Jo-1（患病率 40%）和抗 PMScl 抗体（29.2%）。预定义的低阳性阈值（>10 信号强度）对 IIM-ILD 的鉴别能力最高（AUC 0.86）。将 MA-LIA 与年龄、性别、临床 CTD 表现以及 HRCT 上重叠的非特异性间质性肺炎/机化性肺炎模式相结合，可提高对 IIM-ILD 的鉴别能力（AUC 0.96）。