title: Intensive immunosuppressive therapy improves pulmonary hemodynamics and long-term prognosis in patients with pulmonary arterial hypertension associated with connective tissue disease.
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) remains a serious disease characterized by elevated pulmonary artery pressure (PAP) and increased pulmonary vascular resistance (PVR). Among its subtypes, PAH associated with connective tissue disease (CPAH) has the worse prognosis, because of resistance to conventional vasodilator therapy. We hypothesized that intensive immunosuppressive therapy (IIT) could improve the pulmonary hemodynamics in CPAH.
  
  
  RESULTS
  In our pulmonary hypertension (PH) cohort of 182 patients, we evaluated 13 consecutive patients with CPAH who received IIT combined with cyclophosphamide and glucocorticosteroids (IIT group, mean age 45 ± 8 years, 12 females and 1 male). We compared them with 8 historical controls (control group: mean age 52 ± 18 years, 8 females) for pulmonary hemodynamics and prognosis. Both groups were treated with conventional vasodilator therapy. Although the mean PAP (mPAP) remained unchanged in the control group, IIT significantly decreased mPAP (40 ± 9 to 29 ± 11 mmHg, P < 0.01) and tended to decrease PVR (700 ± 434 to 481 ± 418 dyne·s·cm⁻⁵, P=0.07). Importantly, in 6 of the 13 patients in the IIT group, mPAP was almost normalized (< 25 mmHg) and remained stabilized for more than 1 year. Furthermore, the IIT group showed significantly better prognosis compared with the control group (P<0.01).
  
  
  CONCLUSIONS
  These results suggest that IIT as well as conventional vasodilator therapy improves the pulmonary hemodynamics and long-term prognosis of patients with CPAH.
authors.affiliation: Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, Japan.
authors.forename: Saori
authors.initials: S
authors.lastname: Miyamichi-Yamamoto
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Fukumoto
authors.forename: Koichiro
authors.initials: K
authors.lastname: Sugimura
authors.forename: Tomonori
authors.initials: T
authors.lastname: Ishii
authors.forename: Kimio
authors.initials: K
authors.lastname: Satoh
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Miura
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Tatebe
authors.forename: Kotaro
authors.initials: K
authors.lastname: Nochioka
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Aoki
authors.forename: Zhulanqiqige
authors.initials: Z
authors.lastname: Do E
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Shimokawa
mesh_terms: D000328:Adult; D000368:Aged; D003520:Cyclophosphamide / Q000008:administration & dosage*; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D007165:Immunosuppression Therapy; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D014655:Vascular Resistance / Q000187:drug effects*; D014665:Vasodilator Agents / Q000008:administration & dosage*
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D014665:Vasodilator Agents; D003520:Cyclophosphamide