Nurmi Hanna M, Elfving Pia K, Kettunen Hannu-Pekka, Suoranta Sanna-Katja, Järvinen Henrik M I, Kuittinen Vili A E, Purokivi Minna K, Kaarteenaho Riitta L, Koskela Heikki O
Division of Respiratory Medicine, Institute of Clinical Medicine, University of Eastern Finland, Kuopio, Finland.
Center of Medicine and Clinical Research, Department of Respiratory Medicine, Kuopio University Hospital, Kuopio, Finland.
J Thorac Dis. 2023 Feb 28;15(2):311-322. doi: 10.21037/jtd-22-1219. Epub 2023 Jan 31.
Acute onset of interstitial lung disease (ILD) has been described in patients with idiopathic inflammatory myositis (IIM), but controlled studies about this issue are sparse. The aim of this study was to compare disease onset, demographics, and high-resolution computed tomography (HRCT) patterns in IIM-ILD and other connective tissue disease (CTD)-ILDs.
Clinical and radiological data of 22 IIM-ILD and 132 other CTD-ILD patients was retrospectively gathered from hospital registries between January 2000 and November 2019. Data was re-assessed and compared using a multivariate analysis.
Compared to other CTD-ILDs, the patients with IIM-ILD were younger (59.7 68.0 years, P=0.023), more often non-smokers (71.4% 45.7%, P=0.029) and displayed radiological nonspecific interstitial pneumonia/organizing pneumonia (NSIP/OP) overlap pattern more frequently (27.3% 1.5%, P<0.001). The onset of ILD was acute with patients needing intensive care significantly more often in IIM-ILD than in other CTD-ILDs (22.7% 2.3%, P<0.001). In most patients ILD was diagnosed before or simultaneously with IIM presentation unlike in other CTD-ILDs (90.9% 47.7%, P<0.001). In multivariate analysis, NSIP/OP overlap pattern, acute onset disease treated in intensive care unit and ILD preceding or being diagnosed simultaneously with CTD were significantly associated with IIM-ILD. The multivariate model, supplemented with age, had excellent diagnostic performance identifying IIM-ILD [area under curve (AUC) 0.845].
Unlike other CTD-ILDs, IIM-ILD often develops acutely, simultaneously with the systemic disease. Therefore, clinicians should consider IIM-ILD as an option of differential diagnosis in patients with acute ILD and promptly test muscle enzymes as well as comprehensive autoantibody tests.
特发性炎性肌病（IIM）患者中曾有间质性肺疾病（ILD）急性发作的描述，但关于此问题的对照研究较少。本研究旨在比较IIM-ILD与其他结缔组织病（CTD）-ILD的疾病发作情况、人口统计学特征及高分辨率计算机断层扫描（HRCT）模式。
回顾性收集2000年1月至2019年11月期间医院登记的22例IIM-ILD患者和132例其他CTD-ILD患者的临床及放射学数据。采用多变量分析对数据进行重新评估和比较。
与其他CTD-ILD相比，IIM-ILD患者更年轻（59.7岁对68.0岁，P = 0.023），非吸烟者更多（71.4%对45.7%，P = 0.029），且放射学上非特异性间质性肺炎/机化性肺炎（NSIP/OP）重叠模式更常见（27.3%对1.5%，P < 0.001）。ILD发作多为急性，与其他CTD-ILD相比，IIM-ILD患者需要重症监护的情况明显更常见（22.7%对2.3%，P < 0.001）。与其他CTD-ILD不同，大多数患者的ILD在IIM表现之前或同时被诊断（90.9%对47.7%，P < 0.001）。在多变量分析中，NSIP/OP重叠模式、在重症监护病房治疗的急性发作疾病以及在CTD之前或同时被诊断的ILD与IIM-ILD显著相关。补充年龄后的多变量模型在识别IIM-ILD方面具有出色的诊断性能[曲线下面积（AUC）为0.845]。
与其他CTD-ILD不同，IIM-ILD常与系统性疾病同时急性发作。因此，临床医生应将IIM-ILD视为急性ILD患者鉴别诊断的一个选项，并及时检测肌酶以及进行全面的自身抗体检测。
