Skip to main content

Refractory Takayasu arteritis successfully treated with rituximab: case-based review

Abstract

Takayasu arteritis (TAK) is a subtype of the large-vessel vasculitis, affecting the aorta and its major branches. Although T cell-mediated autoimmunity is mainly involved in vascular inflammation, in recent years, accumulating evidence suggests the important role of B cells in the pathogenesis and effectiveness of B-cell-targeted therapy with rituximab (RTX), a chimeric anti-CD20 monoclonal antibody in refractory TAK. Herein, we report for the first time a case involving a 34-year-old man with TAK who was refractory to four different biologic agents, such as one selective T-cell co-stimulation modulator (abatacept), one anti-interleukin-6 receptor monoclonal antibody (tocilizumab), and two tumor necrosis factor-α inhibitors (infliximab and etanercept), but eventually achieved remission with RTX. He received a total of six courses of RTX, and doses of prednisolone and methotrexate were tapered without relapse. The current case provided further evidence to the potential role of RTX therapy in patients with refractory TAK, and its efficacy needs to be validated in a controlled trial.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2
Fig. 3

References

  1. Kermani TA, Dasgupta B (2018) Current and emerging therapies in large-vessel vasculitis. Rheumatology (Oxford) 57:1513–1524. https://doi.org/10.1093/rheumatology/kex385

    CAS  Article  Google Scholar 

  2. Arnaud L, Haroche J, Mathian A, Gorochov G, Amoura Z (2011) Pathogenesis of Takayasu’s arteritis: a 2011 update. Autoimmun Rev 11:61–67. https://doi.org/10.1016/j.autrev.2011.08.001

    CAS  Article  PubMed  Google Scholar 

  3. Hoyer BF, Mumtaz IM, Loddenkemper K et al (2012) Takayasu arteritis is characterised by disturbances of B cell homeostasis and responds to B cell depletion therapy with rituximab. Ann Rheum Dis 71:75–79. https://doi.org/10.1136/ard.2011.153007

    CAS  Article  PubMed  Google Scholar 

  4. Inder SJ, Bobryshev YV, Cherian SM et al (2000) Immunophenotypic analysis of the aortic wall in Takayasu’s arteritis: involvement of lymphocytes, dendritic cells and granulocytes in immuno-inflammatory reactions. Cardiovasc Surg 8:141–148

    CAS  Article  Google Scholar 

  5. Mackay F, Schneider P, Rennert P, Browning J (2003) BAFF AND APRIL: a tutorial on B cell survival. Annu Rev Immunol 21:231–264

    CAS  Article  Google Scholar 

  6. Nishino Y, Tamai M, Kawakami A et al (2010) Serum levels of BAFF for assessing the disease activity of Takayasu arteritis. Clin Exp Rheumatol 28:14–17

    PubMed  Google Scholar 

  7. Wang H, Ma J, Wu Q, Luo X, Chen Z, Kou L (2011) Circulating B lymphocytes producing autoantibodies to endothelial cells play a role in the pathogenesis of Takayasu arteritis. J Vasc Surg 53:174–180. https://doi.org/10.1016/j.jvs.2010.06.173

    Article  PubMed  Google Scholar 

  8. Hadjadj J, Canaud G, Mirault T et al (2018) mTOR pathway is activated in endothelial cells from patients with Takayasu arteritis and is modulated by serum immunoglobulin G. Rheumatology (Oxford) 57:1011–1020. https://doi.org/10.1093/rheumatology/key017

    CAS  Article  PubMed  Google Scholar 

  9. Galarza C, Valencia D, Tobon GJ et al (2008) Should rituximab be considered as the first-choice treatment for severe autoimmune rheumatic diseases? Clin Rev Allergy Immunol 34:124–128. https://doi.org/10.1007/s12016-007-8028-z

    CAS  Article  PubMed  Google Scholar 

  10. Ernst D, Greer M, Stoll M, Meyer-Olson D, Schmidt RE, Witte T (2012) Remission achieved in refractory advanced takayasu arteritis using rituximab. Case Rep Rheumatol 2012:406963. https://doi.org/10.1155/2012/406963

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  11. Walters HM, Aguiar CL, Macdermott EJ et al (2013) Takayasu arteritis presenting in the context of active tuberculosis: a pediatric case. J Clin Rheumatol 19:344–347. https://doi.org/10.1097/RHU.0b013e31829ce750

    Article  PubMed  Google Scholar 

  12. Caltran E, Di Colo G, Ghigliotti G et al (2014) Two Takayasu arteritis patients successfully treated with rituximab. Clin Rheumatol 33:1183–1184. https://doi.org/10.1007/s10067-014-2506-5

    CAS  Article  PubMed  Google Scholar 

  13. Ahmed M, Mansoor S, Assad S et al (2016) Refractory case of Takayasu arteritis in a young woman: a clinical challenge. Cureus 8(11):e872. https://doi.org/10.7759/cureus.872

    Article  PubMed  PubMed Central  Google Scholar 

  14. O’Connor MB, O’Donovan N, Bond U, Phelan MJ (2017) Successful treatment of Takayasu arteritis with rituximab as a first-line immunosuppressant. BMJ Case Rep 10:2017. https://doi.org/10.1136/bcr-2016-217313

    Article  Google Scholar 

  15. Pazzola G, Muratore F, Pipitone N et al (2018) Rituximab therapy for Takayasu arteritis: a seven patients experience and a review of the literature. Rheumatology (Oxford) 57:1151–1155. https://doi.org/10.1093/rheumatology/kex249

    CAS  Article  Google Scholar 

  16. Nakagomi D, Kronbichler A, Witte T, Mohammad AJ, Jayne DRW (2018) Comment on: rituximab therapy for Takayasu arteritis: a seven patients experience and a review of the literature. Rheumatology (Oxford) 57:1309–1310. https://doi.org/10.1093/rheumatology/kex493

    Article  Google Scholar 

  17. JCS Joint Working Group (2011) Guideline for management of vasculitis syndrome (JCS 2008). Japanese circulation society. Circ J 75:474–503

    Article  Google Scholar 

  18. Kerr GS, Hallahan CW, Giordano J et al (1994) Takayasu arteritis. Ann Intern Med 120:919–929

    CAS  Article  Google Scholar 

  19. Clifford A, Hoffman GS (2014) Recent advances in the medical management of Takayasu arteritis: an update on use of biologic therapies. Curr Opin Rheumatol 26:7–15. https://doi.org/10.1097/BOR.0000000000000004

    CAS  Article  PubMed  Google Scholar 

  20. Gasparyan AY, Ayvazyan L, Blackmore H, Kitas GD (2011) Writing a narrative biomedical review: considerations for authors, peer reviewers, and editors. Rheumatol Int 31:1409–1417

    Article  Google Scholar 

  21. Curtis JR, Westfall AO, Allison J et al (2006) Population-based assessment of adverse events associated with long-term glucocorticoid use. Arthritis Rheum 55:420–426. https://doi.org/10.1002/art.21984

    Article  PubMed  Google Scholar 

  22. Mukhtyar C, Guillevin L, Cid MC et al (2009) EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 68:318–323. https://doi.org/10.1136/ard.2008.088351

    CAS  Article  PubMed  Google Scholar 

  23. Decker P, Olivier P, Risse J, Zuily S, Wahl D (2018) Tocilizumab and refractory Takayasu disease: four case reports and systematic review. Autoimmun Rev 17:353–360. https://doi.org/10.1016/j.autrev.2017.11.026

    CAS  Article  PubMed  Google Scholar 

Download references

Acknowledgements

We would like to thank Editage (www.editage.jp) for English language editing.

Funding

None.

Author information

Authors and Affiliations

Authors

Contributions

TM provided patient’s management, performed the literature review, and wrote the manuscript; TI, KA, YF, YS, and HF were involved in the clinical management; TI, TS, YK, HS, and HH provided intellectual contributions; TI supervised the entire process from drafting the paper to submission of the final manuscript.

Corresponding author

Correspondence to Tomonori Ishii.

Ethics declarations

Conflict of interest

The authors declare that they have no conflicts of interest.

Informed consent

Written informed consent was obtained from this patient.

Additional information

Publisher's Note

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

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Mutoh, T., Ishii, T., Shirai, T. et al. Refractory Takayasu arteritis successfully treated with rituximab: case-based review. Rheumatol Int 39, 1989–1994 (2019). https://doi.org/10.1007/s00296-019-04390-w

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-019-04390-w

Keywords

  • Takayasu arteritis
  • Large-vessel vasculitis
  • Rituximab
  • B cells