Clinicoserological features of antisynthetase syndrome (ASyS)-associated interstitial lung disease presenting to respiratory services: comparison with idiopathic pulmonary fibrosis and ASyS diagnosed in rheumatology services
Introduction Antisynthetase syndrome (ASyS) is a rare autoimmune connective tissue disease (CTD), associated with autoantibodies targeting tRNA synthetase enzymes, that can present to respiratory (interstitial lung disease (ILD)) or rheumatology (myositis, inflammatory arthritis and systemic feature...
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BMJ Publishing Group
2021-01-01
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| Series: | BMJ Open Respiratory Research |
| Online Access: | https://bmjopenrespres.bmj.com/content/8/1/e000829.full |
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| author | Harsha Gunawardena Huzaifa I Adamali Havra H Adamali Caroline Cotton Ben Mulhearn Hina Iftikhar John David Pauling Lisa Spencer |
| author_facet | Harsha Gunawardena Huzaifa I Adamali Havra H Adamali Caroline Cotton Ben Mulhearn Hina Iftikhar John David Pauling Lisa Spencer |
| author_sort | Harsha Gunawardena |
| collection | DOAJ |
| description | Introduction Antisynthetase syndrome (ASyS) is a rare autoimmune connective tissue disease (CTD), associated with autoantibodies targeting tRNA synthetase enzymes, that can present to respiratory (interstitial lung disease (ILD)) or rheumatology (myositis, inflammatory arthritis and systemic features) services. The therapeutic management of CTD-associated ILD and idiopathic pulmonary fibrosis (IPF) differs widely, thus accurate diagnosis is essential.Methods We undertook a retrospective, multicentre observational cohort study designed to (1) evaluate differences between ASyS-associated ILD with IPF, (2) phenotypic differences in patients with ASyS-ILD presenting to respiratory versus rheumatology services, (3) differences in outcomes between ASySassociated with Jo-1 versus non-Jo-1 autoantibodies and (4) compare long-term outcomes between these groups.Results We identified 76 patients with ASyS-ILD and 78 with IPF. Patients with ASyS were younger at presentation (57 vs 77 years, p<0.001) with a female predominance (57% vs 33%, p=0.006) compared with IPF. Cytoplasmic staining on indirect immunofluorescence was a differentiating factor between ASyS and IPF (71% vs 0%, p<0.0001). Patients with ASyS presenting initially to respiratory services (n=52) had a higher prevalence of ASyS non-Jo-1 antibodies and significantly fewer musculoskeletal symptoms/biochemical evidence of myositis, compared with those presenting to rheumatology services (p<0.05), although lung physiology was similar in both groups. There were no differences in high-resolution CT appearances or outcomes in those with Jo-1 versus non-Jo-1 ASyS-ILD.Conclusions Extended autoimmune serology is needed to evaluate for ASyS autoantibodies in patients presenting with ILD, particularly in younger female patients. Musculoskeletal involvement is common in ASyS (typically Jo-1 autoantibodies) presenting to rheumatology but the burden of ILD is similar to those presenting to respiratory medicine. |
| format | Article |
| id | doaj-art-0eecd7ede77842d9baf0098383a62c74 |
| institution | Kabale University |
| issn | 2052-4439 |
| language | English |
| publishDate | 2021-01-01 |
| publisher | BMJ Publishing Group |
| record_format | Article |
| series | BMJ Open Respiratory Research |
| spelling | doaj-art-0eecd7ede77842d9baf0098383a62c742024-11-24T17:30:08ZengBMJ Publishing GroupBMJ Open Respiratory Research2052-44392021-01-018110.1136/bmjresp-2020-000829Clinicoserological features of antisynthetase syndrome (ASyS)-associated interstitial lung disease presenting to respiratory services: comparison with idiopathic pulmonary fibrosis and ASyS diagnosed in rheumatology servicesHarsha Gunawardena0Huzaifa I Adamali1Havra H Adamali2Caroline Cotton3Ben Mulhearn4Hina Iftikhar5John David Pauling6Lisa Spencer7Department of Rheumatology, North Bristol NHS Trust, Bristol, UKBristol Interstitial Lung Disease Service, North Bristol NHS Trust, Bristol, UKBristol Interstitial Lung Disease Service, North Bristol NHS Trust, Bristol, UKLiverpool Interstitial Lung Disease Service, Liverpool University Hospitals NHS Foundation Trust, Liverpool, UKUniversity of Manchester, Arthritis Research UK Centre for Genetics and Genomics, Manchester, United KingdomBristol Interstitial Lung Disease Service, North Bristol NHS Trust, Bristol, UKDepartment of Rheumatology, Royal National Hospital for Rheumatic Diseases, Bath, Somerset, UKLiverpool Interstitial Lung Disease Service, Liverpool University Hospitals NHS Foundation Trust, Liverpool, UKIntroduction Antisynthetase syndrome (ASyS) is a rare autoimmune connective tissue disease (CTD), associated with autoantibodies targeting tRNA synthetase enzymes, that can present to respiratory (interstitial lung disease (ILD)) or rheumatology (myositis, inflammatory arthritis and systemic features) services. The therapeutic management of CTD-associated ILD and idiopathic pulmonary fibrosis (IPF) differs widely, thus accurate diagnosis is essential.Methods We undertook a retrospective, multicentre observational cohort study designed to (1) evaluate differences between ASyS-associated ILD with IPF, (2) phenotypic differences in patients with ASyS-ILD presenting to respiratory versus rheumatology services, (3) differences in outcomes between ASySassociated with Jo-1 versus non-Jo-1 autoantibodies and (4) compare long-term outcomes between these groups.Results We identified 76 patients with ASyS-ILD and 78 with IPF. Patients with ASyS were younger at presentation (57 vs 77 years, p<0.001) with a female predominance (57% vs 33%, p=0.006) compared with IPF. Cytoplasmic staining on indirect immunofluorescence was a differentiating factor between ASyS and IPF (71% vs 0%, p<0.0001). Patients with ASyS presenting initially to respiratory services (n=52) had a higher prevalence of ASyS non-Jo-1 antibodies and significantly fewer musculoskeletal symptoms/biochemical evidence of myositis, compared with those presenting to rheumatology services (p<0.05), although lung physiology was similar in both groups. There were no differences in high-resolution CT appearances or outcomes in those with Jo-1 versus non-Jo-1 ASyS-ILD.Conclusions Extended autoimmune serology is needed to evaluate for ASyS autoantibodies in patients presenting with ILD, particularly in younger female patients. Musculoskeletal involvement is common in ASyS (typically Jo-1 autoantibodies) presenting to rheumatology but the burden of ILD is similar to those presenting to respiratory medicine.https://bmjopenrespres.bmj.com/content/8/1/e000829.full |
| spellingShingle | Harsha Gunawardena Huzaifa I Adamali Havra H Adamali Caroline Cotton Ben Mulhearn Hina Iftikhar John David Pauling Lisa Spencer Clinicoserological features of antisynthetase syndrome (ASyS)-associated interstitial lung disease presenting to respiratory services: comparison with idiopathic pulmonary fibrosis and ASyS diagnosed in rheumatology services BMJ Open Respiratory Research |
| title | Clinicoserological features of antisynthetase syndrome (ASyS)-associated interstitial lung disease presenting to respiratory services: comparison with idiopathic pulmonary fibrosis and ASyS diagnosed in rheumatology services |
| title_full | Clinicoserological features of antisynthetase syndrome (ASyS)-associated interstitial lung disease presenting to respiratory services: comparison with idiopathic pulmonary fibrosis and ASyS diagnosed in rheumatology services |
| title_fullStr | Clinicoserological features of antisynthetase syndrome (ASyS)-associated interstitial lung disease presenting to respiratory services: comparison with idiopathic pulmonary fibrosis and ASyS diagnosed in rheumatology services |
| title_full_unstemmed | Clinicoserological features of antisynthetase syndrome (ASyS)-associated interstitial lung disease presenting to respiratory services: comparison with idiopathic pulmonary fibrosis and ASyS diagnosed in rheumatology services |
| title_short | Clinicoserological features of antisynthetase syndrome (ASyS)-associated interstitial lung disease presenting to respiratory services: comparison with idiopathic pulmonary fibrosis and ASyS diagnosed in rheumatology services |
| title_sort | clinicoserological features of antisynthetase syndrome asys associated interstitial lung disease presenting to respiratory services comparison with idiopathic pulmonary fibrosis and asys diagnosed in rheumatology services |
| url | https://bmjopenrespres.bmj.com/content/8/1/e000829.full |
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