Background:Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are major causes of death in systemic sclerosis (SSc). Six-minute-walk test (6MWT) is a standard outcome measure for exercise capacity in cardiopulmonary diseases. However, the results of 6MWT may not reflect real cardiopulmonary function of SSc patients in whom musculoskeletal system is frequently inflicted.Objectives:This study aimed to evaluate the clinical utility of breath-holding test (BHT) in evaluating cardiopulmonary function in SSc patients, as compared with 6MWT.Methods:Seventy-two patients with SSc were prospectively enrolled and underwent BHT and 6MWT with measurement of Borg score and Scleroderma Health Assessment Questionnaire (SHAQ). Data on diffusing capacity for carbon monoxide (DLCO, %), forced vital capacity (FVC, % and liters), and ejection fraction and pulmonary arterial systolic pressure (PASP) measured by transthoracic echocardiography (TTE), were also collected. For BHT, participants were required to make a maximum expiration followed by a maximum inspiration and to hold the breath as long as possible at maximum inspiratory level. This procedure was repeated three times, with 5-minute intervals. 6MWT was performed according to the American Thoracic Society guidelines. Pearson’s correlation test was applied to demonstrate the relationship between BHT results and each clinical parameter.Results:Among 72 (66 female) patients, mean (SD) age was 57.1 (11.1) years, modified Rodnan skin score 10.6 (10.5), SHAQ 0.64 (0.61) and 6MWT distance 473.5 (95.5) m. Mean BHT time was 35.05 (14.90) sec at the first time, 38.92 (16.14) sec at the second time, and 41.11 (17.71) sec at the third time. The BHT time showed a statistically significant negative correlation with Borg scale (pre-test, r = -0.336, p = 0.002; post-test, r = -0.252, p = 0.034; Figure 1 and Table 1), while 6MWT showed a negative correlation with only post-test Borg scale (pre-test, r = -0.113 p = 0.343; post-test, r = -0.351 p = 0.002; Table 1). The BHT time was positively correlated with DLCO (%, r = 0.409, p < 0.001) and FVC (liters, r = 0.402, p < 0.001) (Table 1). We also found a statistically significant correlation between BHT time and SHAQ score (r = -0.451, p < 0.001; Table 1). However, EF and PASP by TTE showed no significant relationship with BHT time (EF, r = -0.108, p = 0.374; PASP, r = -0.246, p = 0.054; Table 1).Table 1.Pearson’s correlation coefficients (r) for the relation between BHT and clinical parameters in comparison to 6MWT.Pre-test Borg scalePost-test Borg scaleDLCO(%)FVC(L)FVC(%)FVC/DLCOEF(%)PSAP(mm Hg)SHAQ (score)BHT (sec)-0.366**-0.252*0.409***0.402**0.191-0.244***-0.108-0.246-0.451***6MWT (m)-0.113-0.351**0.297*0.321**0.063-0.250*0.137-0.354**-0.531***BHT, breath-holding test; 6MWT, 6-minute-walk test; DLCO, diffusing capacity for carbon monoxide; FVC, forced vital capacity; EF, ejection fraction estimated by transthoracic echocardiography; SHAQ, Scleroderma Health Assessment Questionnaire.* p < 0.05, ** p < 0.01, *** p < 0.001Figure 1.Association of Borg dyspnea scale with breath-holding time.Conclusion:The BHT is a simple, safe, and less time-consuming test, reflective of pulmonary parameters and SHAQ, as compared with 6MWT. Our results suggest that the BHT might be a useful surrogate marker of cardiopulmonary capacity in SSc patients.References:[1]Villalba WO, Sampaio-Barros PD, Pereira MC, Cerqueira EM, Leme CA, Jr., Marques-Neto JF, et al. Six-minute walk test for the evaluation of pulmonary disease severity in scleroderma patients. Chest. 2007;131(1):217-22.[2]Garin MC, Highland KB, Silver RM, Strange C. Limitations to the 6-minute walk test in interstitial lung disease and pulmonary hypertension in scleroderma. J Rheumatol. 2009;36(2):330-6.[3]Barnai M, Laki I, Gyurkovits K, Angyan L, Horvath G. Relationship between breath-hold time and physical performance in patients with cystic fibrosis. Eur J Appl Physiol. 2005;95(2-3):172-8.Acknowledgements:This study would not have been possible without help from research assistant, Sung-Soon Cho.Disclosure of Interests:Jina Yeo: None declared, Mi Hyeon Kim: None declared, Jun Won Park: None declared, Jin Kyun Park: None declared, Eun Bong Lee Consultant of: Pfizer, Grant/research support from: GC Pharma and Handok Inc.

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BackgroundThe Idiopathic Inflammatory Myositis (IIM) are heterogeneous with different clinical profiles associated with various myositis autoantibodies. Among the Myositis specific antibodies (MSA), antibodies against aminoacyl-tRNA synthetases(ARS) is related to the clinically unique IIM subsets, Antisynthetase syndrome(ASS).ObjectivesThe aim of this study was to identify the prevalence and clinical features of patients with ARS autoantibodies compared with other myositis antibodies in a single center cohort of IIM in Korea.MethodsAdult patients diagnosed with IIM and positive for myositis antibodies were recruited in the myositis cohort from January 2020 to December 2022. Retrospective analysis was performed based on the clinical data including clinical features, laboratory data, high-resolution CT (HRCT) findings, and pulmonary function manifestations.ResultsAmong 82 patients, 25 patients were positive for ARS autoantibodies and 57 patients had other myositis antibodies. The most common ARS antibody was anti-EJ (n=10) followed by anti-PL-7 (n=8). MSA other than ARS antibodies included anti-SRP (n=6), anti-TIF1γ (n=11) and anti-MDA-5(n=19). Anti-Ro52 was detected in 48 patients and 79.2% of which were accompanied by other antibodies.The most common clinical manifestations of patients with ARS antibodies were respiratory symptoms, more commonly experienced than patients with other myositis antibodies (84% vs. 47.4%, p=0.002). Meanwhile, muscle weakness (32.0% vs. 61.4%) and dysphagia (8.0% vs. 33.3%) were less frequently exhibited compared with those with other myositis antibodies. In regard to laboratory findings, the ARS antibodies positive group showed higher inflammatory markers than other myositis antibodies group, the mean (SD) of erythrocyte sedimentation rate (ESR) was 53.6 (34.6) and 33.8 (24.3), respectively. Anti-Ro52 and anti-nuclear antibodies (ANA) cytoplasmic pattern were more detected in the ARS antibodies positive group. Interstitial lung disease (ILD) was also more commonly detected in patients with ARS antibodies than other myositis antibodies group (88.0% vs. 59.6%) and non-specific interstitial pneumonia (NSIP) was the most frequent pattern of ILD. However, there were no significant differences in the baseline pulmonary function between the two groups.ConclusionPatients with ARS antibodies experienced more respiratory symptoms while muscle weakness is less common clinical manifestations than patients with other myositis antibodies. Moreover, higher inflammatory markers and ANA cytoplasmic pattern along with Anti-Ro52 were more commonly detected in the ARS antibodies positive group.References[1]DA SILVA, Lila Morena Bueno, et al. Demographic, clinical, laboratory data, prognostic, and treatment features of patients with antisynthetase syndrome: An international, two-center cohort study. Archives of Rheumatology, 2022, 37.3: 424-434.[2]GUPTA, Latika, et al. Myositis-specific and myositis-associated autoantibodies in a large Indian cohort of inflammatory myositis. In: Seminars in Arthritis and Rheumatism. WB Saunders, 2021. p. 113-120.Table 1.Baseline characteristics of patients with ARS antibodies compared to those with other myositis antibodies (n=82)ARS Ab(+) (n=25)Other myositis Ab(+) (n=57)PClinical feature, n (%)DOE21 (84.0)27 (47.4)0.002Weakness8 (32.0)35 (61.4)0.014Dysphagia2 (8.0)19 (33.3)0.016Fever7 (28.0)8 (14.0)0.132Mechanic’s hand11 (44.0)17 (29.8)0.213Raynaud phenomenon8 (32.0)16 (28.1)0.719Arthritis7 (28.0)13 (22.8)0.614Laboratory findingsANA, cytoplasmic Ab(+)9 (36.0)7 (13.0)0.014Ro5222 (88.0)26 (45.6)<0.001ESR53.56 ± 34.5933.78 ± 24.170.014CRP1.90± 2.190.76 ± 1.620.028PFTFVC72.0 ± 16.373.3 ± 18.90.772FEV175.5 ± 14.181.5 ± 21.00.152DLCO62.6 ± 21.962.8 ± 23.00.969ILD, n(%)22 (88.0)34 (59.6)0.011NSIP17 (70.8)22 (44.9)0.037Acknowledgements:NIL.Disclosure of InterestsNone Declared.