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, Koushan Kouranloo Department of Rheumatology, University Hospital Lewisham , Lewisham High St, London SE13 6LH School of Medicine, Cedar House, University of Liverpool , Ashton Street, Liverpool, U.K., L69 3GE Corresponding author Koushan Kouranloo, k.kouranloo2@liverpool.ac.uk, Department of Rheumatology, University Hospital Lewisham, Lewisham High St, London SE13 6LH. Search for other works by this author on: Oxford Academic Mrinalini Dey Centre for Rheumatic Diseases, Kings’ College London, Weston Education Centre , Cutcombe Rd., London, U.K., SE5 9RJ Search for other works by this author on: Oxford Academic Helen Elwell British Medical Association library, BMA House , Tavistock Square, London, U.K., WC1H 9JP Search for other works by this author on: Oxford Academic Veronica Yioe Liverpool Regional Interstitial Lung Disease Service, Aintree Chest Centre, Aintree Hospital, Liverpool University NHS Foundation Trust , Lower Lane, Liverpool, U.K., L9 7AL Search for other works by this author on: Oxford Academic Lisa G Spencer Liverpool Regional Interstitial Lung Disease Service, Aintree Chest Centre, Aintree Hospital, Liverpool University NHS Foundation Trust , Lower Lane, Liverpool, U.K., L9 7AL Search for other works by this author on: Oxford Academic Caroline V Cotton Department of Rheumatology, Liverpool University NHS Foundation Trust , Mount Vernon St, Liverpool, U.K., L7 8YE Search for other works by this author on: Oxford Academic
Rheumatology, keae403, https://doi.org/10.1093/rheumatology/keae403
Published:
31 July 2024
Article history
Received:
23 March 2024
Revision received:
11 July 2024
Accepted:
16 July 2024
Published:
31 July 2024
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Koushan Kouranloo, Mrinalini Dey, Helen Elwell, Veronica Yioe, Lisa G Spencer, Caroline V Cotton, Management and outcomes of interstitial lung disease associated with anti-synthetase syndrome: A systematic literature review, Rheumatology, 2024;, keae403, https://doi.org/10.1093/rheumatology/keae403
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Abstract
Background
Anti-synthetase syndrome (ASS) is a chronic autoimmune condition, with interstitial lung disease (ILD) being a key feature. This systematic literature review (SLR; CRD42023416414) aimed to summarise treatments and outcomes of ILD associated with ASS (ASS-ILD).
Methods
Databases were searched for articles discussing ASS-ILD management and outcomes, published 1946-September 2023.
Screening and data-extraction were performed by two reviewers. Meta-analysis, using a random effects model, and paired t-tests, were undertaken where appropriate to evaluate post-treatment-change in Pulmonary Function Tests (PFT).
Results
Ten articles were included, comprising 514 patients: 67.8% female, mean age 52.4years (SD4.6). Baseline high-resolution computed tomography was documented in 447 patients (86.9%); the most common pattern was non-specific interstitial pneumonia (n = 220; 49.2%). The most common myositis-associated autoantibody was anti-Jo1 (48%) with 27.8% having associated anti-Ro52 antibodies.
Pooled estimates, after meta-analysis, for baseline Forced Vital Capacity (FVC) was 60.8% predicted (SE 2.1), and Diffusion Capacity of Lungs for Carbon Monoxide (DLco) was 49.8% (SE 3.5). After one-year, pooled improvement in FVC was 14.1% from baseline (SE 3.1) and in DLco was 15.1% (SE 2.8). Paired t-test demonstrated significant overall improvement in FVC (p = 0.007) and DLco (p = 0.002).
Patients receiving RTX had 12.2% improvement in FVC and 2.9% increase in DLco at one-year; for patients receiving CYC, there was 17% improvement and 6.3% increase, respectively. 28 deaths were reported.
Conclusion
Our SLR, the first to summarise management and outcomes of ASS-ILD, found no conclusive difference between effectiveness of treatments. More robust trials are required to reduce morbidity and mortality resulting from ASS-ILD.
anti-synthetase syndrome, interstitial lung disease, myositis, CTD-ILD, connective tissue disease
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