Background Antisynthetase Syndrome (ASSD) is a chronic autoimmune condition characterized by antibodies directed against an aminoacycl transfer RNA synthetase (ARS) along with a group of clinical features including the classical clinical triad: inflammatory myopathy, arthritis, interstitial lung disease (ILD). ASSD is highly heterogenous due to the different organs involvement, and ILD is the main cause of mortality and function loss, which presents as different patterns when diagnosed. We designed this retrospective cohort to describe the clinical features and disease behavior of the ASSD) associated ILD. Methods Retrospectively collected the data of 108 cases of ASSD associated ILD in Beijing Chaoyang Hospital since 2017.12 to 2019.3. Data including age, gender, physical examination, laboratory test, pulmonary function and High Resolution Computer Tomography (HRCT), treatment, were obtained from the Electronic Medical Record (EMR) system. Patients were divided into 5 groups according to the distinct Aminoacyl tRNA synthetases (ARS) antibodies, and all patients had a multiple discussion team (MDT) to make a radiological and pathological diagnosis of the ILD pattern. Each patient had at least 1 follow up for no less than 6 months. Patients with missing data of MDT evaluation or follow up were excluded. Results: 108 consecutive patients were recruited in this retrospective cohort. 30 cases received bronchoscopy for a transbronchial lung biopsy. 3 had bronchoscopy for a transbronchial cryobiopsy. 33 were with JO-1 positive and 30 were with PL-7 positive. 23 with EJ positive, 13 with PL12 positive and 9 with OJ positive. JO-1 group had a significant higher rate of mechanic’s hand (57.6%) than other 4 groups, the skin involvement (Gottron Papules and/or Heliotrope Rash) was found in 38 (35%) and no difference was found among the 5 groups. Polymyositis/Dermatomyositis (PM/DM) was diagnosed in 25 (23.1%) patients and no difference was observed among the 5 groups. The anti-PL7 positive group had a higher frequency of UIP pattern (13.3%) than other 4 groups but the difference was not significant due to the small sample size, and EJ group had the most frequent OP pattern(78.2%), which was significantly higher than PL-7 group (P<0.001) and PL-12 group (P=0.025). The median follow-up time were 10.7 months. All received prednisone treatment, with or without immunosuppressants ,and at the 6-month-follow up, the JO-1 group and EJ group had the significantly higher improvement of forced vital capacity that the other 3 groups (P<0.05), and PL-7 group had the lowest FVC improvement (P<0.05). The anti-Jo1-positive group and anti-EJ-positive group had significantly higher anti-RO52-positive occurrence than other 3 groups (P<0.05).Conclusion Anti PL-7 antibody had the same frequency as anti-JO1 in ASSD-ILD, in which the ILD pattern was different with distinct anti-ARS antibodies. All ASSD-ILD responded to therapy of steroids, with or without immunosuppressants. PL-7 group had a highest occurrence of UIP pattern, and significantly lower respondence to therapy.

Background: Anti-synthetase syndrome (ASSD) is a chronic autoimmune condition characterized by antibodies directed against an aminoacycl transfer RNA synthetase (ARS) along with a group of clinical features including the classical clinical triad: inflammatory myopathy, arthritis, and interstitial lung disease (ILD). ASSD is highly heterogenous due to different organ involvement, and ILD is the main cause of mortality and function loss, which presents as different patterns when diagnosed. We designed this retrospective cohort to describe the clinical features and disease behaviour of ASSD associated ILD. Methods: Data of ASSD associated ILD were retrospectively collected in Beijing Chaoyang Hospital from 2017.12 to 2019.3. Data were obtained from the Electronic Medical Record (EMR) system. Patients were divided into 5 groups according to distinct aminoacyl tRNA synthetase (ARS) antibodies. Results: 108 consecutive patients were recruited. 33 were JO-1 positive,30 were PL-7 positive,23 were EJ positive, 13 were PL12 positive and 9 were OJ positive. The JO-1 group had a significant higher rate of mechanic's hand (57.6%) than other 4 groups. Polymyositis/ dermatomyositis (PM/DM) was diagnosed in 25 (23.1%) patients and no difference was observed among the 5 groups. The PL7 group had a higher frequency of UIP pattern (13.3%) than the other 4 groups but the difference was not significant, and the EJ group had the most frequent OP pattern(78.2%), which was significantly higher than PL7 (P<0.001) and PL12 groups (P=0.025). The median follow-up time was 10.7 months. All received prednisone treatment, with or without immunosuppressants. At 6-month-follow up, 96.3% (104/108) had a positive response to therapy, the JO1 and EJ groups had a significantly higher improvement of forced vital capacity (FVC) than the other 3 groups (P<0.05), and PL-7 group had the lowest FVC improvement (P<0.05). The JO1 and EJ groups had significantly higher anti-RO52-positive occurrence than the other 3 groups (P<0.05). Conclusion: Anti PL-7 antibody had the same frequency as anti-JO1 in ASSD-ILD, while the ILD pattern was different with distinct anti-ARS antibodies. Most ASSD-ILD had a positive response to steroids, with or without immunosuppressants. The PL-7 group had the highest occurrence of UIP pattern, and a significantly lower response to therapy.

BackgroundAntisynthetase Syndrome (ASSD) is a chronic autoimmune condition characterized by antibodies directed against an aminoacycl transfer RNA synthetase (ARS) along with a group of clinical features including the classical clinical triad: inflammatory myopathy, arthritis, interstitial lung disease (ILD). ASSD is highly heterogenous due to the different organs involvement, and ILD is the main cause of mortality and function loss, which presents as different patterns when diagnosed. We designed this retrospective cohort to describe the clinical features and disease behavior of the ASSD associated ILD. MethodsRetrospectively collected the data of 108 cases of ASSD associated ILD in Beijing Chaoyang Hospital since 2017.12 to 2019.3. Data was obtained from the Electronic Medical Record (EMR) system. Patients were divided into 5 groups according to the distinct Aminoacyl tRNA synthetases (ARS) antibodies. Results108 consecutive patients were recruited. 33 were with JO-1 positive and 30 were with PL-7 positive. 23 with EJ positive, 13 with PL12 positive and 9 with OJ positive. JO-1 group had a significant higher rate of mechanic’s hand (57.6%) than other 4 groups. Polymyositis/ Dermatomyositis (PM/DM) was diagnosed in 25 (23.1%) patients and no difference was observed among the 5 groups. The anti-PL7 positive group had a higher frequency of UIP pattern (13.3%) than other 4 groups but the difference was not significant, and EJ group had the most frequent OP pattern(78.2%), which was significantly higher than PL-7 group (P<0.001) and PL-12 group (P=0.025). The median follow-up time were 10.7 months. All received prednisone treatment, with or without immunosuppressants, and at the 6-month-follow up, the JO-1 group and EJ group had a significantly higher improvement of forced vital capacity that the other 3 groups (P<0.05), and PL-7 group had the lowest FVC improvement (P<0.05). The anti-Jo1-positive group and anti-EJ-positive group had significantly higher anti-RO52-positive occurrence than other 3 groups (P<0.05).ConclusionAnti PL-7 antibody had the same frequency as anti-JO1 in ASSD-ILD, in which the ILD pattern was different with distinct anti-ARS antibodies. All ASSD-ILD responded to therapy of steroids, with or without immunosuppressants. PL-7 group had a highest occurrence of UIP pattern, and significantly lower respondence to therapy.