Abstract
We report the case of a 69-year-old Japanese woman who presented with thrombotic thrombocytopenic purpura (TTP) which had manifested soon after the onset of adult-onset Still’s disease (AOSD). Her disease was multi-drug resistant. She had undergone treatment with high-dose glucocorticoids, two courses of steroid pulse therapy, and cyclosporine A. The patient initially had a favorable response to the administration of etanercept (an anti-tumor necrosis factor agent) and glucocorticoids. However, her disease became refractory to etanercept after 6 months. Therefore, we administered tocilizumab (a humanized monoclonal anti-IL-6 receptor antibody) which dramatically improved the patient’s refractory AOSD with TTP. This is the first report of an effective treatment for AOSD with TTP using the biological agents. Our report strongly suggests that biological agents, especially a humanized monoclonal anti-IL-6 receptor antibody, may be a new option for a safe and effective treatment of multi-drug-resistant AOSD and TTP associated with AOSD.
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References
Efthimiou P, Paik PK, Bielory L (2006) Diagnosis and management of adult onset Still’s disease. Ann Rheum Dis 65:564–572
Saiki O, Uda H, Nishimoto N et al (2004) Adult Still’s disease reflects a Th2 rather than a Th1 cytokine profile. Clin Immunol 112:120–125
Yamaguchi M, Ohta A, Tsunematsu T et al (1992) Preliminary criteria for classification of adult Still’s disease. J Rheumatol 19:424–431
Bakshi R, Shaikh ZA, Bates VE et al (1999) Thrombotic thrombocytopenic purpura: brain CT and MRI findings in 12 patients. Neurology 52:1285–1288
Fautrel B, Sibilia J, Mariette X et al (2005) Tumour necrosis factor alpha blocking agents in refractory adult Still’s disease: an observational study of 20 cases. Ann Rheum Dis 64:262–266
Radstake TR, Svenson M, Eijsbouts AM et al (2009) Formation of antibodies against infliximab and adalimumab strongly correlates with functional drug levels and clinical responses in rheumatoid arthritis. Ann Rheum Dis 68:1739–1745
Fitzgerald AA, Leclercq SA, Yan A et al (2005) Rapid responses to anakinra in patients with refractory adult-onset Still’s disease. Arthritis Rheum 52:1794–1803
Matsumoto K, Nagashima T, Takatori S et al (2009) Glucocorticoid and cyclosporine refractory adult onset Still’s disease successfully treated with tocilizumab. Clin Rheumatol 28:485–487
De Bandt M, Saint-Marcoux B (2009) Tocilizumab for multirefractory adult-onset Still’s disease. Ann Rheum Dis 68:153–154
Ruggenenti P, Noris M, Remuzzi G (2001) Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. Kidney Int 60:831–846
Furlan M, Robles R, Galbusera M et al (1998) Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 339:1578–1584
Sayarlioglu M, Sayarlioglu H, Ozkaya M et al (2008) Thrombotic thrombocytopenic purpura–hemolytic uremic syndrome and adult onset Still’s disease: case report and review of the literature. Mod Rheumatol 18:403–406
George JN (2006) Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med 354:1927–1935
Masson C, Myhal D, Menard H et al (1986) Purpura thrombotique thrombopenique fatal chez une patiente atteinte dune maladie de Still de I’adulte. Rev Rhum Mal Osteoartric 53:389–391
Domingues RB, da Gama AM, Caser EB et al (2003) Disseminated cerebral thrombotic microangiopathy in a patient with adult Still’s disease. Arq Neuropsiquiatr 61:259–261
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Sumida, K., Ubara, Y., Hoshino, J. et al. Etanercept-refractory adult-onset Still’s disease with thrombotic thrombocytopenic purpura successfully treated with tocilizumab. Clin Rheumatol 29, 1191–1194 (2010). https://doi.org/10.1007/s10067-010-1418-2
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DOI: https://doi.org/10.1007/s10067-010-1418-2