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International Journal of Rheumatology
Volume 2010, Article ID 720305, 8 pages
Review Article

Interleukin-6 as a Potential Therapeutic Target for Pulmonary Arterial Hypertension

1Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi,Shinjuku-ku, Tokyo 160-8582, Japan
2Department of Cardiology, Kyorin University Graduate School of Medicine, Mitaka, Tokyo 181-8611, Japan

Received 13 May 2010; Accepted 6 July 2010

Academic Editor: Oliver Distler

Copyright © 2010 Yoshiaki Furuya et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Interleukin-6 (IL-6) is a pleiotropic cytokine with a wide range of biologic activities in immune regulation, hematopoiesis, inflammation, and oncogenesis. Recent accumulating evidence indicates a pathologic role for IL-6 in promoting proliferation of both smooth muscle and endothelial cells in the pulmonary arterioles, resulting in development of pulmonary arterial hypertension (PAH). Here, we describe a patient with mixed connective tissue disease and severe, refractory PAH. Her functional activity and hemodynamic parameters dramatically responded to tocilizumab, a humanized monoclonal antibody to human IL-6 receptor, which was aimed at treating multicentric Castleman's disease. It appears that IL-6 blockade may hold promise as an adjunct drug in treatment of PAH in idiopathic form as well as in association with connective tissue disease.