Skip to main content

Advertisement

SpringerLink
Log in

Treatment of refractory adult-onset still’s disease with tocilizumab: report of two cases and review of the literature

  • Short Communication
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

Adult-onset Still’s disease (AOSD) is empirically treated with nonsteroidal anti-inflammatory drugs, corticosteroids, conventional disease-modifying antirheumatic drugs, tumor necrosis factor–blocking agents or anakinra. The monoclonal anti-interleukin (IL)-6 antibody tocilicumab (TOC) has recently been approved for the treatment of rheumatoid arthritis and may be an attractive therapeutic option for AOSD as well. We report two AOSD patients treated with TOC and review of the current data on the use of TOC in AOSD. TOC was applied to the first patient after failure of cloroquin, methotrexate, adalimumab and etanercept. The second patient received TOC because of inefficacious methotrexate treatment. TOC was well tolerated by both the patients, and no clinically significant side effects occurred. Including these two cases, a total of seven AOSD patients have been successfully treated with TOC so far. TOC may be a promising treatment option for AOSD patients refractory to conventional disease-modifying antirheumatic drugs anakinra and tumor necrosis factor-\(\tilde{\alpha }\).

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

References

  1. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H et al (1992) Preliminary criteria for classification of adult Still’s disease. J Rheumatol 19(3):424–430

    PubMed  CAS  Google Scholar 

  2. Colina M, Govoni M, Trotta F (2009) Fatal myocarditis in adult-onset Still disease with diffuse intravascular coagulation. Rheumatol Int 29(11):1355–1357

    Article  PubMed  Google Scholar 

  3. Thonhofer R, Soleiman A, Kriessmayr M, Thonhofer U, Wipfler E, Gaugg M et al (2006) Decrease of proteinuria in a patient with adult-onset Still’s disease and glomerulonephritis after anti-TNFalpha therapy. Scand J Rheumatol 35(6):485–488

    Article  PubMed  CAS  Google Scholar 

  4. Fitzgerald AA, Leclercq SA, Yan A, Homik JE, Dinarello CA (2005) Rapid responses to anakinra in patients with refractory adult-onset Still’s disease. Arthritis Rheum 52(6):1794–1803

    Article  PubMed  CAS  Google Scholar 

  5. Fautrel B, Sibilia J, Mariette X, Combe B (2005) Tumour necrosis factor alpha blocking agents in refractory adult Still’s disease: an observational study of 20 cases. Ann Rheum Dis 64(2):262–266

    Article  PubMed  CAS  Google Scholar 

  6. Yang DH, Chang DM, Lai JH, Kuo SY, Ho TY, Lin KM et al (2008) Etanercept as a rescue agent in patient with adult onset Still’s disease complicated with congestive heart failure. Rheumatol Int 29(1):95–98

    Article  PubMed  CAS  Google Scholar 

  7. Ohsugi Y (2007) Recent advances in immunopathophysiology of interleukin-6: an innovative therapeutic drug, tocilizumab (recombinant humanized anti-human interleukin-6 receptor antibody), unveils the mysterious etiology of immune-mediated inflammatory diseases. Biol Pharm Bull 30(11):2001–2006

    Article  PubMed  CAS  Google Scholar 

  8. Iwamoto M, Nara H, Hirata D, Minota S, Nishimoto N, Yoshizaki K (2002) Humanized monoclonal anti-interleukin-6 receptor antibody for treatment of intractable adult-onset Still’s disease. Arthritis Rheum 46(12):3388–3389

    Article  PubMed  CAS  Google Scholar 

  9. Efthimiou P, Paik PK, Bielory L (2006) Diagnosis and management of adult onset Still’s disease. Ann Rheum Dis 65(5):564–572

    Article  PubMed  CAS  Google Scholar 

  10. Scheinberg MA, Chapira E, Fernandes ML, Hubscher O (1996) Interleukin 6: a possible marker of disease activity in adult onset Still’s disease. Clin Exp Rheumatol 14(6):653–655

    PubMed  CAS  Google Scholar 

  11. Matsumoto K, Nagashima T, Takatori S, Kawahara Y, Yagi M, Iwamoto M et al (2009) Glucocorticoid and cyclosporine refractory adult onset Still’s disease successfully treated with tocilizumab. Clin Rheumatol 28(4):485–487

    Article  PubMed  Google Scholar 

  12. De Bandt M, Saint-Marcoux B (2009) Tocilizumab for multirefractory adult-onset Still’s disease. Ann Rheum Dis 68(1):153–154

    Article  PubMed  Google Scholar 

  13. Cunha ML, Wagner J, Osawa A, Scheinberg M (2010) The effect of tocilizumab on the uptake of 18FDG-PET imaging in patients with adult-onset Still’s disease. Rheumatology (Oxford) 49(5):104–106

    Google Scholar 

  14. Fattori E, Cappelletti M, Costa P, Sellitto C, Cantoni L, Carelli M et al (1994) Defective inflammatory response in interleukin 6-deficient mice. J Exp Med 180(4):1243–1250

    Article  PubMed  CAS  Google Scholar 

  15. Chai Z, Gatti S, Toniatti C, Poli V, Bartfai T (1996) Interleukin (IL)-6 gene expression in the central nervous system is necessary for fever response to lipopolysaccharide or IL-1 beta: a study on IL-6-deficient mice. J Exp Med 183(1):311–316

    Article  PubMed  CAS  Google Scholar 

  16. Iwamoto M (2007) Macrophage activation syndrome associated with adult-onset Still’s disease. Nihon Rinsho Meneki Gakkai Kaishi 30(6):428–431

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Internal Medicine, State Hospital Muerzzuschlag, Grazer Strasse 63-65, 8680, Muerzzuschlag, Austria

    Rene Thonhofer, Manuela Hiller, Markus Trummer & Cornelia Siegel

  2. Department of Rheumatology, Medical University Graz, Graz, Austria

    Rene Thonhofer & Christian Dejaco

  3. Department of Internal Medicine II, State Hospital Klagenfurt, Klagenfurt, Austria

    Horst Just

Authors
  1. Rene Thonhofer
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Manuela Hiller
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Horst Just
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Markus Trummer
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Cornelia Siegel
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Christian Dejaco
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Rene Thonhofer.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Thonhofer, R., Hiller, M., Just, H. et al. Treatment of refractory adult-onset still’s disease with tocilizumab: report of two cases and review of the literature. Rheumatol Int 31, 1653–1656 (2011). https://doi.org/10.1007/s00296-010-1631-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-010-1631-y

Keywords

Search

Navigation