Hui Liu1, Sheng Xie2, Tian Liang1, Li Ma3, Hongliang Sun1, Huaping Dai4, Chen Wang5. 1. Department of Radiology, China-Japan Friendship Hospital, Beijing, 100029, China. 2. Department of Radiology, China-Japan Friendship Hospital, Beijing, 100029, China. xs_mri@126.com. 3. Department of Rheumatology, China-Japan Friendship Hospital, Beijing, 100029, China. 4. Department of Pulmonology, Beijing Chaoyang Hospital, Beijing, 100029, China. 5. Department of Pulmonology, China-Japan Friendship Hospital, Beijing, 100029, China.
Abstract
OBJECTIVES: Interstitial lung disease (ILD) is a common extra-muscular manifestation of anti-synthetase syndrome (ASS) and the main cause of morbidity and mortality in patients with ASS. Data on prognostic factors in these patients are lacking. METHODS: A total of 69 patients with ILD and positivity for at least one of the following autoantibodies were included: anti-Jo-1, anti-PL7, anti-PL12, and anti-EJ. Relevant clinical characteristics were registered. According to the changes in the extent of abnormalities at the follow-up on high-resolution computed tomography (HRCT), three groups were defined: the regression, stability, and deterioration groups. Univariate analysis was performed to evaluate possible prognostic factors and multivariate analysis by logistic regression was then applied to determine the independent prognostic factors in ASS-ILD. RESULTS: The cohort comprised 69 patients positive for anti-synthetase antibodies, i.e., 30 for anti-Jo-1, 16 for anti-EJ, 13 for anti-PL7, and 10 for anti-PL12. The mean length of follow-up was 15 months. Sex, age at diagnosis, fever at presentation, and counts of CD3+CD4+ cells were significantly different among the three groups. According to the multivariate analysis, fever at presentation, lower counts of CD3+CD4+ cells, and a pattern of usual interstitial pneumonia were the three independent risk factors for poor outcomes of ASS-ILD. CONCLUSIONS: At the onset of ASS, some clinical features and HRCT pattern of ILD may suggest an unfavorable outcome of lung involvement on HRCT, even with routine therapy. These factors may contribute to the high long-term mortality of ASS. KEY POINTS: • Evaluation of lung involvement on HRCT is important in the follow-up of patients with interstitial lung disease related to anti-synthetase syndrome (ASS-ILD). • The interstitial lung disease related to ASS responds to the treatment variably. • Some clinical and imaging characteristics are associated with poor prognosis in patients with ASS-ILD, including fever at diagnosis, a lower serum CD3 + /CD4 + level, and a UIP pattern.
OBJECTIVES:Interstitial lung disease (ILD) is a common extra-muscular manifestation of anti-synthetase syndrome (ASS) and the main cause of morbidity and mortality in patients with ASS. Data on prognostic factors in these patients are lacking. METHODS: A total of 69 patients with ILD and positivity for at least one of the following autoantibodies were included: anti-Jo-1, anti-PL7, anti-PL12, and anti-EJ. Relevant clinical characteristics were registered. According to the changes in the extent of abnormalities at the follow-up on high-resolution computed tomography (HRCT), three groups were defined: the regression, stability, and deterioration groups. Univariate analysis was performed to evaluate possible prognostic factors and multivariate analysis by logistic regression was then applied to determine the independent prognostic factors in ASS-ILD. RESULTS: The cohort comprised 69 patients positive for anti-synthetase antibodies, i.e., 30 for anti-Jo-1, 16 for anti-EJ, 13 for anti-PL7, and 10 for anti-PL12. The mean length of follow-up was 15 months. Sex, age at diagnosis, fever at presentation, and counts of CD3+CD4+ cells were significantly different among the three groups. According to the multivariate analysis, fever at presentation, lower counts of CD3+CD4+ cells, and a pattern of usual interstitial pneumonia were the three independent risk factors for poor outcomes of ASS-ILD. CONCLUSIONS: At the onset of ASS, some clinical features and HRCT pattern of ILD may suggest an unfavorable outcome of lung involvement on HRCT, even with routine therapy. These factors may contribute to the high long-term mortality of ASS. KEY POINTS: • Evaluation of lung involvement on HRCT is important in the follow-up of patients with interstitial lung disease related to anti-synthetase syndrome (ASS-ILD). • The interstitial lung disease related to ASS responds to the treatment variably. • Some clinical and imaging characteristics are associated with poor prognosis in patients with ASS-ILD, including fever at diagnosis, a lower serum CD3 + /CD4 + level, and a UIP pattern.
Authors: I Marie; S Josse; O Decaux; S Dominique; E Diot; C Landron; P Roblot; S Jouneau; P Y Hatron; K P Tiev; O Vittecoq; D Noel; L Mouthon; J-F Menard; F Jouen Journal: Autoimmun Rev Date: 2012-02-03 Impact factor: 9.754
Authors: Thomas J Richards; Aaron Eggebeen; Kevin Gibson; Samuel Yousem; Carl Fuhrman; Bernadette R Gochuico; Noreen Fertig; Chester V Oddis; Naftali Kaminski; Ivan O Rosas; Dana P Ascherman Journal: Arthritis Rheum Date: 2009-07
Authors: Iago Pinal-Fernandez; Maria Casal-Dominguez; Julio A Huapaya; Jemima Albayda; Julie J Paik; Cheilonda Johnson; Leann Silhan; Lisa Christopher-Stine; Andrew L Mammen; Sonye K Danoff Journal: Rheumatology (Oxford) Date: 2017-06-01 Impact factor: 7.580
Authors: Kerry B Stone; Chester V Oddis; Noreen Fertig; Yasuhiro Katsumata; Mary Lucas; Molly Vogt; Robyn Domsic; Dana P Ascherman Journal: Arthritis Rheum Date: 2007-09
Authors: I Tillie-Leblond; M Wislez; D Valeyre; B Crestani; A Rabbat; D Israel-Biet; M Humbert; L J Couderc; B Wallaert; J Cadranel Journal: Thorax Date: 2007-06-08 Impact factor: 9.139
Authors: Helena Andersson; Trond Mogens Aaløkken; Anne Günther; Georg Karl Mynarek; Torhild Garen; May Brit Lund; Øyvind Molberg Journal: J Rheumatol Date: 2016-04-01 Impact factor: 4.666
Authors: Shaney L Barratt; Havra H Adamali; Caroline Cotton; Ben Mulhearn; Hina Iftikhar; John David Pauling; Lisa Spencer; Huzaifa I Adamali; Harsha Gunawardena Journal: BMJ Open Respir Res Date: 2021-01
Authors: Elena K Joerns; Traci N Adams; Chad A Newton; Bonnie Bermas; David Karp; Kiran Batra; Jose Torrealba; Lesley Davila; Joan Reisch; Craig Glazer; Una E Makris Journal: J Clin Rheumatol Date: 2022-03-01 Impact factor: 3.902