Skip to main content
SpringerLink
Log in

Analysis of the clinical features of antisynthetase syndrome: a retrospective cohort study in China

  • Original Article
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Objective

To summarize the clinical, serological, and radiological characteristics of anti-synthetase syndrome (ASS) patients with different anti-aminoacyl-tRNA synthase antibody.

Methods

Retrospective analysis was performed based on the clinical data of 88 patients diagnosed with ASS in Tianjin Medical University General Hospital from January 2015 to December 2020. The clinical data included general conditions, serological indexes, high-resolution CT (HRCT) characteristics, and pulmonary function manifestations.

Results

Among the 88 patients, there were 17 males and 71 females. The anti-synthetase antibodies included anti-Jo-1 (n = 42), anti-PL-7 (n = 14), anti-PL-12 (n = 9), anti-EJ (n = 20), and anti-OJ (n = 3) antibodies. The most common clinical manifestations of ASS patients were interstitial lung disease (ILD) (90%, 79/88), followed by myositis (79.5%, 70/88), arthritis (50%, 44/88), and rash (50%, 44/88). The frequency of arthritis in the anti-Jo-1 antibody–positive group was higher than that of the anti-PL-7 and anti-EJ antibody groups (P = 0.004, P = 0.002, respectively). The frequency of Gottron’s sign in the anti-PL-7 antibody positive group was higher than that of the anti-Jo-1 and anti-PL-12 antibody–positive groups (P = 0.006, P = 0.04). Isolated arthritis was the most frequent initial symptoms in anti-Jo-1 antibody–positive group (47.6%, 20/42), while isolated ILD was most frequent in patients with anti-EJ antibody (50%, 10/20), and isolated myositis in patients carrying anti-OJ (66.7%, 2/3). There were only 32 cases (36.4%) with the typical clinical triad (myositis, arthritis, ILD). In our cohort, 79 patients (90%) were complicated with ILD. Meanwhile, 7 out of 79 cases were classified into rapid progressive group with 6 cases (85.7%) carrying anti-Ro-52 antibody. The probability of reticular and honeycombing shadow in HRCT of patients with anti-EJ antibody positive was higher than that of other groups (P < 0.05).

Conclusion

ILD, myositis, and arthritis were the most common clinical manifestations in patients with ASS. Different antibody-positive patients have different initial symptoms. Patients with isolated arthritis, myositis, and ILD should be vigilant of ASS. The complication of anti-Ro-52 antibody in ASS patients was associated with rapidly progressive pulmonary interstitial disease. Patients with positive anti-EJ antibodies tend to have ILD as the first symptom, and with high occurrence of ILD, the HRCT showed more serious patterns, suggesting the correlation between anti-EJ antibodies and ILD.

Key Points

• Analyzing specific clinical manifestations in ASS patients with different ARS antibodies can raise awareness of the disease and reduce misdiagnosis.

• Anti-EJ antibodies were correlated with ILD.

• Anti-Ro-52 antibodies may correlate with RP-ILD in ASS patients.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Data availability

The datasets used and analysed in the present research are available from the corresponding author on reasonable request.

References

  1. Mahler M, Miller F, Fritzler M (2014) Idiopathic inflammatory myopathies and the anti-synthetase syndrome: a comprehensive review [J]. Autoimmunity Rev 13(0):367–371

  2. Imbert-Masseau A, Hamidou M, Agard C et al (2003) Antisynthetase syndrome [J]. Joint Bone Spine 70(3):161–168

    Article  PubMed  Google Scholar 

  3. Satoh M, Tanaka S, Ceribelli A et al (2017) A comprehensive overview on myositis-specific antibodies: new and old biomarkers in idiopathic inflammatory myopathy [J]. Clin Rev Allergy Immunol 52(1):11–19

    Article  Google Scholar 

  4. McHugh N, Tansley S (2018) Autoantibodies in myositis [J]. Nat Rev Rheumatol 14(5):290–302

    Article  CAS  PubMed  Google Scholar 

  5. Witt L, Curran J, Strek M (2016) The diagnosis and treatment of antisynthetase syndrome [J]. Clin Pulm Med 23(5):218–226

    Article  PubMed  PubMed Central  Google Scholar 

  6. Travis W, Costabel U, Hansell D et al (2013) An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias [J]. Am J Respir Crit Care Med 188(6):733–748

    Article  PubMed  PubMed Central  Google Scholar 

  7. Gono T, Kawaguchi Y, Satoh T et al (2010) Clinical manifestation and prognostic factor in anti-melanoma differentiation-associated gene 5 antibody-associated interstitial lung disease as a complication of dermatomyositis [J]. Rheumatology (Oxford) 49(9):1713–1719

    Article  CAS  PubMed  Google Scholar 

  8. Zhan X, Yan W, Wang Y et al (2021) Clinical features of anti-synthetase syndrome associated interstitial lung disease: a retrospective cohort in China [J]. BMC Pulm Med 21(1):57–61

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Shi J, Li S, Yang H et al (2017) Clinical profiles and prognosis of patients with distinct antisynthetase autoantibodies [J]. J Rheumatol 44(7):1051–1057

    Article  CAS  PubMed  Google Scholar 

  10. La Corte R, Lo Mo naco A, Locaputo A et al (2006) In patients with antisynthetase syndrome the occurrence of anti-Ro/SSA antibodies causes a more severe interstitial lung disease [J]. Autoimmunity 39(3):249–253

    Article  PubMed  Google Scholar 

  11. Váncsa A, Csípo I, Németh J et al (2009) Characteristics of interstitial lung disease in SS-A positive/Jo-1 positive inflammatory myopathy patients [J]. Rheumatol Int 29(9):989–994

    Article  PubMed  Google Scholar 

  12. Cavagna L, Trallero-Araguás E, Meloni F et al (2019) Influence of antisynthetase antibodies specificities on antisynthetase syndrome clinical spectrum time course [J]. J Clin Med 8(11)

  13. Aggarwal R, Cassidy E, Fertig N et al (2014) Patients with non-Jo-1 anti-tRNA-synthetase autoantibodies have worse survival than Jo-1 positive patients [J]. Ann Rheum Dis 73(1):227–232

    Article  PubMed  Google Scholar 

  14. Kumar R, Jha S, Dhooria A et al (2021) Anti-Jo-1 syndrome often misdiagnosed as rheumatoid arthritis (for many years): a single-center experience [J]. J Clin Rheumatol Pract Rep Rheum Musculoskelet Dis 27(4):150–155

    Google Scholar 

  15. Andersson H, Aaløkken T, Günther A et al (2016) Pulmonary involvement in the antisynthetase syndrome: a comparative cross-sectional Study [J]. J Rheumatol 43(6):1107–1113

    Article  PubMed  Google Scholar 

  16. Johnson C, Connors G, Oaks J et al (2014) Clinical and pathologic differences in interstitial lung disease based on antisynthetase antibody type [J]. Respir Med 108(10):1542–1548

    Article  CAS  PubMed  Google Scholar 

  17. Gasparotto M, Gatto M, Saccon F et al (2019) Pulmonary involvement in antisynthetase syndrome [J]. Curr Opin Rheumatol 31(6):603–610

    Article  CAS  PubMed  Google Scholar 

  18. Chen F, Zuo Y, Li S et al (2019) Clinical characteristics of dermatomyositis patients with isolated anti-Ro-52 antibody associated rapid progressive interstitial lung disease: data from the largest single Chinese center [J]. Respir Med 155:127–132

  19. Pinal-Fernandez I, Casal-Dominguez M, Huapaya J et al (2017) A longitudinal cohort study of the anti-synthetase syndrome: increased severity of interstitial lung disease in black patients and patients with anti-PL7 and anti-PL12 autoantibodies [J]. Rheumatology (Oxford) 56(6):999–1007

    Article  CAS  PubMed  Google Scholar 

  20. Zhang Y, Ge Y, Yang H et al (2020) Clinical features and outcomes of the patients with anti-glycyl tRNA synthetase syndrome [J]. Clin Rheumatol 39(8):2417–2419

    Article  PubMed  Google Scholar 

  21. Marie I, Hatron P, Dominique S et al (2012) Short-term and long-term outcome of anti-Jo1-positive patients with anti-Ro52 antibody [J]. Semin Arthritis Rheum 41(6):890–899

    Article  CAS  PubMed  Google Scholar 

  22. Shao C, Sun Y, Huang H et al (2021) Myositis specific antibodies are associated with isolated anti-Ro-52 associated interstitial lung disease [J]. Rheumatology (Oxford, England) 61(3):1083–1091

Download references

Acknowledgements

Not applicable.

Author information

Author notes

  1. Ruoming Wang and Yin Zhao contributed equally to this work.

Authors and Affiliations

  1. Department of Rheumatology and Immunology, Tianjin First Central Hospital, Tianjin, 300192, People’s Republic of China

    Ruoming Wang

  2. Department of Rheumatology and Immunology, Tianjin Medical University General Hospital, 300052, Tianjin, People’s Republic of China

    Yin Zhao, Fumin Qi, Xiuhua Wu, Yingai Wang, Yong Xu, Yanan Wu, Na Zhang, Hou Hou, WenWen Sun, Xin Li & Wei Wei

Authors
  1. Ruoming Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Yin Zhao
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Fumin Qi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Xiuhua Wu
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Yingai Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Yong Xu
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Yanan Wu
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Na Zhang
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Hou Hou
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. WenWen Sun
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Xin Li
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Wei Wei
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Wei Wei.

Ethics declarations

Ethics

Ethics approval was waived by the IRB (Institutional Review Board) of Tianjin Medical University General Hospital.

Competing interests

The authors declare that they have no competing interests.

Additional information

Publisher's note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Wang, R., Zhao, Y., Qi, F. et al. Analysis of the clinical features of antisynthetase syndrome: a retrospective cohort study in China. Clin Rheumatol 42, 703–709 (2023). https://doi.org/10.1007/s10067-022-06404-8

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-022-06404-8

Keywords

Search

Navigation