Heart and Vessels

, Volume 25, Issue 5, pp 444–447

The efficacy of tocilizumab in a patient with pulmonary arterial hypertension associated with Castleman’s disease

Authors

  • Yoh Arita
    • Department of Cardiovascular MedicineOsaka University Graduate School of Medicine
  • Yasushi Sakata
    • Department of Cardiovascular MedicineOsaka University Graduate School of Medicine
  • Takao Sudo
    • Department of Hematology and OncologyOsaka University Graduate School of Medicine
  • Tetsuo Maeda
    • Department of Hematology and OncologyOsaka University Graduate School of Medicine
  • Ken Matsuoka
    • Department of Cardiovascular MedicineOsaka University Graduate School of Medicine
  • Keito Tamai
    • Department of Cardiovascular MedicineOsaka University Graduate School of Medicine
  • Kaori Higuchi
    • Department of Cardiovascular MedicineOsaka University Graduate School of Medicine
  • Wataru Shioyama
    • Department of Cardiovascular MedicineOsaka University Graduate School of Medicine
  • Yoshikazu Nakaoka
    • Department of Cardiovascular MedicineOsaka University Graduate School of Medicine
  • Yuzuru Kanakura
    • Department of Hematology and OncologyOsaka University Graduate School of Medicine
    • Department of Cardiovascular MedicineOsaka University Graduate School of Medicine
Case Report

DOI: 10.1007/s00380-009-1215-5

Cite this article as:
Arita, Y., Sakata, Y., Sudo, T. et al. Heart Vessels (2010) 25: 444. doi:10.1007/s00380-009-1215-5

Abstract

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 words

Pulmonary arterial hypertensionInterleukin-6CytokineInterleukin-6 receptor

Copyright information

© Springer Japan 2010