The efficacy of tocilizumab in a patient with pulmonary arterial hypertension associated with Castleman's disease.
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.
Arita
Yoh
Y
Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine, Suita, Japan.
Sakata
Yasushi
Y
Sudo
Takao
T
Maeda
Tetsuo
T
Matsuoka
Ken
K
Tamai
Keito
K
Higuchi
Kaori
K
Shioyama
Wataru
W
Nakaoka
Yoshikazu
Y
Kanakura
Yuzuru
Y
Yamauchi-Takihara
Keiko
K
D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D005871:Castleman Disease / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D013256:Steroids / Q000627:therapeutic use*; D013997:Time Factors; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D000959:Antihypertensive Agents; D013256:Steroids; C502936:tocilizumab