The efficacy of tocilizumab in a patient with pulmonary arterial hypertension associated with Castleman’s disease
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Castleman’s disease is a highly heterogeneous clinical-pathological entity that belongs to the lymphoproliferative disorders and is associated with pulmonary arterial hypertension (PAH) in some patients. It is linked to excessive immune stimulation by interleukin-6 (IL-6), which is also involved in the pathogenesis of PAH. A 31-year-old woman with Castleman’s disease demonstrated PAH characterized by severe right heart failure. Since she was resistant to various conventional therapies including steroids, prostacyclins, bosentan, and sildenafil, tocilizumab (anti-IL-6 receptor antibody) therapy was started. Her clinical course was followed for 6 months, with significant improvement without any adverse effect. This is the first reported case of use of tocilizumab in addition to steroids and conventional PAH therapy in a patient with PAH associated with Castleman’s disease.
Key wordsPulmonary arterial hypertension Interleukin-6 Cytokine Interleukin-6 receptor
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- 5.Nishimoto N, Kanakura Y, Aozasa K, Johkoh T, Nakamura M, Nakano S, Nakano N, Ikeda Y, Sasaki T, Nishioka K, Hara M, Taguchi H, Kimura Y, Kato Y, Asaoku M, Kumagai S, Kodama F, Nakahara H, Hagihara K, Yoshizaki K, Kishimoto T (2005) Humanized anti-interleukin-6 receptor antibody treatment of multicentric Castleman disease. Blood 106:2627–2632CrossRefPubMedGoogle Scholar
- 7.Nishimoto N, Yoshizaki K, Miyasaka N, Yamamoto K, Kawai S, Takeuchi T, Hashimoto J, Azuma J, Kishimoto T (2004) Treatment of rheumatoid arthritis with humanized anti-interleukin-6 receptor antibody: a multicenter, double-blind, placebo-controlled trial. Arthritis Rheum 50:1761–1769CrossRefPubMedGoogle Scholar
- 12.American Thoracic Society (2002) ATS statement guidelines for the six-minute walk test. Am J Respir Crit Care Med 166:111–117Google Scholar
- 17.Ito T, Okada T, Miyashita H, Nomoto T, Nonaka-Sarukawa M, Uchibori R, Maeda Y, Urabe M, Mizukami H, Kume A, Takahashi M, Ikeda U, Shimada K, Ozawa K (2007) Interleukin-10 expression mediated by an adeno-associated virus vector prevents monocrotaline-induced pulmonary arterial hypertension in rats. Circ Res 101:734–741CrossRefPubMedGoogle Scholar
- 18.Nishimoto N, Terao K, Mima T, Nakahara H, Takagi N, Kakehi T (2008) Mechanisms and pathologic significances in increase in serum interleukin-6 (IL-6) and soluble IL-6 receptor after administration of an anti-IL-6 receptor antibody, tocilizumab, in patients with rheumatoid arthritis and Castleman disease. Blood 112:3959–3964CrossRefPubMedGoogle Scholar