Pos0068 changes in quantitative interstitial lung disease scores on high-resolution ct in idiopathic inflammatory myositis

Background The idiopathic inflammatory myopathies (IIM) are autoimmune connective tissue diseases affecting skeletal muscle, skin and other organ systems. IIM-related interstitial lung disease (IIM-ILD) is the most common extra-muscular manifestation, being the leading cause of morbidity and mortality. Several studies have suggested that ILD pattern based on chest high-resolution computed tomography (HRCT) can be related to disease course and treatment response, but the results vary considerably. Moreover, the clinical impact of the quantitative ILD (QILD) score, a validated computer-aided scoring system in assessing ILD severity from HRCT, and its longitudinal changes have not yet been evaluated in IIM-ILD. Objectives This study aims to investigate ILD patterns and QILD scores in patients with IIM-ILD, to identify their clinical impact, and to delineate longitudinal changes of QILD measurement. Methods A total of 80 patients with IIM (polymyositis 22, and dermatomyositis 58) who underwent at least 2 times of serial HRCT scans were included. Visual ILD patterns were assessed by multiple thoracic radiologists. Quantitative analysis of HRCT was presented as total extent of QILD scores (%) in whole lung and most severe zone. Individual time-estimated ΔQILD score between first 2 visits was derived using a linear approximation of yearly change, where the duration of median (IQR) was 1.0 (0.4-1.6) years in the first 2 HRCT scans. Results The median (IQR) age of the patients was 52.0 (43.5-58.5) years and 60 (75.0%) were women. Baseline median score of whole lung-QILD and most severe zone-QILD were 28.1% (19.1-43.8) and 68.0% (45.5-81.8), respectively, and QILD score showed significant correlations with pulmonary function tests (r=-0.349, p=0.002 for % predicted forced vital capacity; and r=-0.381, p=0.001 for % predicted diffusing capacity for carbon monoxide). The individual time-estimated yearly ΔQILD score between first 2 visits presented that approximately half of the patients showed improvement or stability in QILD scores; however, when patients were sorted by visual assessment in ILD subtype on HRCT, approximately two-thirds of the patients with usual interstitial pneumonia (UIP) pattern were aggravated in QILD scores and less than half of subjects with nonspecific interstitial pneumonia and organizing pneumonia were aggravated (Figure 1, 80% for UIP vs. 44.4% for non-UIP, p=0.013). There was no immunosuppressive drugs related to meaningful improvement in QILD scores during first 2 visits. Notably, we observed significant aggravation of QILD scores in tacrolimus users (n=7, median time-estimated whole lung-yarly ΔQILD 20.3 (2.7-38.4)) compared with tacrolimus non-users (n=73, median time estimated whole lung-yearly ΔQILD -1.2 (-8.3-6.5)). Among 80 patients, 6 (7.5%) were died due to various lung complications. Higher baseline QILD scores were noted in deaths (median whole lung-QILD 45.4 (32.9-56.5)) than in survivors (median whole lung-QILD 26.9 (19.0-42.4)), albeit not significant (p=0.084). Poor survival rate was observed in patients with high grade of ground glass opacity by visual assessment in right upper lobe (log-rank test, p=0.042). Among subgroup of patients with 3 serial HRCT scans (n=41), dynamic changes of four distinct patterns (improving, worsening, convex, and concave) were observed. Figure 1. Cleveland dot plot of individual time-estimated yearly ΔQILD during fist 2 visits. Conclusion The changes in QILD score in IIM-ILD are dynamic and present different by visual assessment. QILD score has the potential for evaluation of the severity changes, prognosis and medication response in patients with IIM-ILD. References [1]Tashkin DP, et al. Ann Rheum Dis 2016;75(2):374-81. 7 truncated values in the graph A. NSIP: nonspecific interstitial pneumonia; OP: organizing pneumonia; UIP: usual interstitial pneumonia. Disclosure of Interests None declared