TY - JOUR
T1 - Diversity of borderline pulmonary arterial pressure associated with systemic sclerosis
T2 - 3 case series
AU - Sugawara, Eri
AU - Kato, Masaru
AU - Sato, Takahiro
AU - Takahashi, Kenta
AU - Kono, Michihiro
AU - Hisada, Ryo
AU - Kono, Michihito
AU - Oku, Kenji
AU - Bohgaki, Toshiyuki
AU - Yasuda, Shinsuke
AU - Tsujino, Ichizo
AU - Tanaka, Shinya
AU - Atsumi, Tatsuya
N1 - Publisher Copyright:
© 2016, © 2016 Japan College of Rheumatology.
PY - 2017/1/2
Y1 - 2017/1/2
N2 - Mean pulmonary arterial pressure of 21 to 24 mmHg, called borderline pulmonary arterial pressure (BoPAP), may represent a pre-pulmonary arterial hypertension (PAH) condition but has not so far been indicated for specific PAH therapy. Despite the recent progress in PAH-targeted therapy, the prognosis of patients with systemic sclerosis (SSc)-associated PAH still remains poor. One of the possible strategies to improve the prognosis of those patients is the early detection and therapeutic intervention of pre-PAH conditions. We present three different clinical courses of BoPAP associated with SSc, which include successful treatment with bosentan and tocilizumab, an improvement following the treatment of interstitial lung disease, and spontaneous progression to fatal PAH, and suggest the efficacy of frequent follow-up examinations and therapeutic intervention for BoPAP in SSc patients.
AB - Mean pulmonary arterial pressure of 21 to 24 mmHg, called borderline pulmonary arterial pressure (BoPAP), may represent a pre-pulmonary arterial hypertension (PAH) condition but has not so far been indicated for specific PAH therapy. Despite the recent progress in PAH-targeted therapy, the prognosis of patients with systemic sclerosis (SSc)-associated PAH still remains poor. One of the possible strategies to improve the prognosis of those patients is the early detection and therapeutic intervention of pre-PAH conditions. We present three different clinical courses of BoPAP associated with SSc, which include successful treatment with bosentan and tocilizumab, an improvement following the treatment of interstitial lung disease, and spontaneous progression to fatal PAH, and suggest the efficacy of frequent follow-up examinations and therapeutic intervention for BoPAP in SSc patients.
KW - borderline pulmonary arterial pressure
KW - interstitial lung disease
KW - Pulmonary arterial hypertension
KW - systemic sclerosis
KW - tocilizumab
UR - http://www.scopus.com/inward/record.url?scp=85040968335&partnerID=8YFLogxK
U2 - 10.1080/24725625.2016.1259722
DO - 10.1080/24725625.2016.1259722
M3 - 学術論文
AN - SCOPUS:85040968335
SN - 2472-5625
VL - 1
SP - 9
EP - 14
JO - Modern Rheumatology Case Reports
JF - Modern Rheumatology Case Reports
IS - 1
ER -