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Defining anti-synthetase syndrome: a systematic literature review


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23

 

  1. Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
  2. Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
  3. School of Medicine and Surgery, University of Milano-Bicocca, Milan, Italy.
  4. Center for Interstitial and Rare Lung Diseases, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Germany.
  5. University of Sydney, Australia.
  6. Brigham and Women's Hospital, Boston, MA, USA.
  7. Department of Dermatology, Stanford University School of Medicine, Redwood City, CA, USA.
  8. Hospital Universitario Marques de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
  9. Department of Medicine, McGill University, Montreal, Quebec, Canada.
  10. Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.
  11. Division of Rheumatology, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden.
  12. National Institute of Arthritis and Musculoskeletal and Skin Disorders, National Institutes of Health, Bethesda, MD, and Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  13. University of Bath, UK.
  14. Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, USA.
  15. Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
  16. Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
  17. Interstitial Lung Disease and Rheumatology Units, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, México City, México.
  18. Department of Neurology, University Medical Center Göttingen; Department of Neurology and Pain Treatment, Immanuel Klinik Rüdersdorf, University Hospital of the Brandenburg Medical School Theodor Fontane, Rüdersdorf bei Berlin; and Faculty of Health Sciences Brandenburg, Brandenburg Medical School Theodor Fontane, Rüdersdorf bei Berlin, Germany.
  19. Vall d'Hebron Hospital, Universitat Autònoma de Barcelona, Spain.
  20. University of Pennsylvania, Perelman School of Medicine and Michael J. Crescenz VAMC, Philadelphia, PA, USA.
  21. Istituti Clinici Scientifici Maugeri, University of Pavia, Italy. filiciasak@gmail.com
  22. Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
  23. Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.

CER15311
2022 Vol.40, N°2
PI 0309, PF 0319
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PMID: 35225224 [PubMed]

Received: 05/11/2021
Accepted : 03/12/2021
In Press: 25/02/2022
Published: 25/02/2022

Abstract

OBJECTIVES:
Anti-synthetase syndrome (ASSD) is a heterogeneous autoimmune disease characterised by multi-system involvement with a wide variety of manifestations. Validated classification criteria are necessary to improve recognition and prevent misclassification, especially given the lack of reliable and standardised autoantibody testing. We systematically reviewed the literature to analyse proposed ASSD criteria, characteristics, and diagnostic performance.
METHODS:
We searched PubMed and Embase databases (01/01/1984 to 06/11/2018) and the ACR and EULAR meeting abstracts (2017-2018). Sensitivities, specificities, positive, negative likelihood ratios and risk of bias were calculated for ASSD criteria and key variables reported in the literature. We performed meta-analysis when appropriate.
RESULTS:
We retrieved 4,358 studies. We found 85 proposed ASSD criteria from a total of 82 studies. All but one study included anti-synthetase autoantibody (ARS) positivity in the ASSD criteria. Most studies required only one ASSD feature plus anti-ARS to define ASSD (n=64, 78%), whereas 16 studies required more than one ASSD variable plus anti-ARS. The only criteria not including anti-ARS positivity required 5 ASSD clinical features. We found limited data and wide variability in the diagnostic performance of each variable and definition proposed in the literature. Given these limitations we only meta-analysed the performance of individual muscle biopsy and clinical variables in diagnosing ASSD, which performed poorly.
CONCLUSIONS:
The current ASSD criteria include a variety of serological, clinical, and histological features with wide variability amongst proposed definitions and the performance of these definitions has not been tested. This systematic literature review suggests the need for additional data and consensus-driven classification criteria for ASSD.

DOI: https://doi.org/10.55563/clinexprheumatol/8xj0b9

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