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Sustained response to tocilizumab in a patient with relapsing polychondritis with aortic involvement: a case based review

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Abstract

This paper presents a case with refractory relapsing polychondritis (RPC), complicated with severe aortic involvement, which is successfully treated with tocilizumab. Previous treatments consisted of methotrexate, corticosteroids, cyclosporine, cyclophosphamide, infliximab, and etanercept. With these treatments, the patient had recurrent episodes of fever, polyarthritis, tenosynovitis, subcutaneous nodules, and progressive cardiac disease. One year after the start of treatment with tocilizumab, there is resolution of all symptoms, normalization of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), and the dose of prednisolone is tapered down to 2 mg/day. We have reviewed the English literature for reports of patients with refractory RPC, successfully treated with tocilizumab. We found five additional case reports. In one case report, a patient with refractory RPC complicated with aortitis was successfully treated with tocilizumab. In three case reports, patients with refractory RPC complicated with laryngotracheal involvement were successfully treated with tocilizumab. All cases had, like our patient, failed conventional treatment. We also reviewed the literature for reports of the effect of biologicals on cardiac involvement in RPC. Current literature is presented and discussed.

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References

  1. Lahmer T, Treiber M, Von Werder A, Foerger F, Knopf A, Heemann U, Thuermel K (2010) Relapsing polychondritis: an autoimmune disease with many faces. Autoimmun Rev 9:540–546. doi:10.1016/j.autrev.2010.02.016

    Article  CAS  PubMed  Google Scholar 

  2. Rapini RP, Warner NB (2006) Relapsing polychondritis. J Clin Dermatol 24:482–485

    Article  Google Scholar 

  3. Zeuner M, Straub RH, Rauh G, Albert ED, Schölmerich J, Lang B (1997) Relapsing polychondritis: clinical and immunogenetic analysis of 62 patients. J Rheumatol 24:96–101

    CAS  PubMed  Google Scholar 

  4. McAdam LP, O’Hanlan MA, Bluestone R, Pearson CM (1976) Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Med (Baltimore) 55(3):193–215

    Article  CAS  Google Scholar 

  5. Kent PD, Michet CJ Jr, Luthra HS (2004) Relapsing polychondritis. Curr Opin Rheumatol 16(1):56–61

    Article  PubMed  Google Scholar 

  6. Kemta Lekpa F, Kraus VB, Chevalier X (2012) Biologics in relapsing polychondritis: a literature review. Semin Arthritis Rheum 41:712–719. doi:10.1016/j.semarthrit.2011.08.006

    Article  PubMed  Google Scholar 

  7. Stabler T, Piette JC, Chevalier X, Marini-Portugal A, Kraus VB (2004) Serum cytokine profiles in relapsing polychondritis suggest monocyte/macrophage activation. Arthritis Rheum 50:3663–3667

    Article  CAS  PubMed  Google Scholar 

  8. Sharma A, Gnanapandithan K, Sharma K, Sharma S (2013) Relapsing polychondritis: a review. Clin Rheumatol 32:1575–1583. doi:10.1007/s10067-013-2328-x

    Article  PubMed  Google Scholar 

  9. Kawai M, Hagihara K, Hirano T, Shima Y, Kuwahara Y, Arimitsu J, Narazaki M, Ogata A, Kawase I, Kishimoto T, Tanaka T (2009) Sustained response to tocilizumab, anti-interleukin-6 receptor antibody, in two patients with refractory relapsing polychondritis. Rheumatology (Oxford) 48:318–331. doi:10.1093/rheumatology/ken468

    Article  CAS  Google Scholar 

  10. Narshi CB, Allard SA (2012) Sustained response to tocilizumab, anti-IL-6 antibody, following anti-TNF-alpha failure in a patient with relapsing polychondritis complicated by aortitis. Rheumatology 51:952–953. doi:10.1093/rheumatology/ker451

    Article  CAS  PubMed  Google Scholar 

  11. Chana JK, Ahmed AA, Edmonds S (2012) Successful use of tocilizumab in a Caucasian patient with relapsing polychondritis complicated by Sweet’s syndrome. Rheumatology 51(suppl 3):iii153–iii154

    Google Scholar 

  12. Wallace ZS, Stone JH (2013) Refractory relapsing polychondritis treated with serial success with interleukin 6 receptor blockade. J Rheumatol 40(1):100–101. doi:10.3899/jrheum.120381

    Article  PubMed  Google Scholar 

  13. Weber E, Gaultier JB, Paul S, Guichard I, Monard E, Cathébras P (2013) Sustained response with tocilizumab in a case of refractory relapsing polychondritis. Rev Med Interne. doi:10.1016/j.revmed.2013.02.019

    PubMed  Google Scholar 

  14. Dib C, Moustafa SE, Mookadam M, Zehr KJ, Michet CJ Jr, Mookadam F (2006) Surgical treatment of the cardiac manifestations of relapsing polychondritis: overview of 33 patients identified through literature review and the mayo clinic records. Mayo Clin Proc 81(6):772–776

    Article  PubMed  Google Scholar 

  15. Marie I, Lahaxe L, Josse S, Levesque H (2009) Sustained response to infliximab in a patient with relapsing polychondritis with aortic involvement. Rheumatology (Oxford) 48:1328–1329. doi:10.1093/rheumatology/kep224

    Article  Google Scholar 

  16. Mekinian A, Lambert M, Beregi JP, Morell-Dubois S, Launay D, Queyrel V, Midulla M, Hachulla E, Hatron PY (2009) Aortic aneurysm in MAGIC syndrome successfully managed with combined anti-TNF-alpha and stent grafting. Rheumatology (Oxford) 48:1169–1170. doi:10.1093/rheumatology/kep172

    Article  Google Scholar 

  17. Seymour MW, Home DM, Williams RO, Allard SA (2007) Prolonged response to anti-tumour necrosis factor treatment with adalimumab (Humira) in relapsing polychondritis complicated by aortitis. Rheumatology (Oxford) 46:1738–1739

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Rheumatology, University Hospital of Ghent, De Pintelaan 185, 9000, Ghent, Belgium

    Rebecca Stael, Vanessa Smith, Ruth Wittoek & Herman Mielants

  2. Department of Pathology, University Hospital of Ghent, De Pintelaan 185, 9000, Ghent, Belgium

    David Creytens

Authors
  1. Rebecca Stael
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  2. Vanessa Smith
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  3. Ruth Wittoek
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  4. David Creytens
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  5. Herman Mielants
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Correspondence to Rebecca Stael.

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Stael, R., Smith, V., Wittoek, R. et al. Sustained response to tocilizumab in a patient with relapsing polychondritis with aortic involvement: a case based review. Clin Rheumatol 34, 189–193 (2015). https://doi.org/10.1007/s10067-014-2670-7

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  • DOI: https://doi.org/10.1007/s10067-014-2670-7

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