Intensive Immunosuppressive Therapy Improves Pulmonary Hemodynamics and Long-Term Prognosis in Patients With Pulmonary Arterial Hypertension Associated With Connective Tissue Disease

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 resist...

Full description

Saved in:
Bibliographic Details
Published in:Circulation Journal 2011, Vol.75(11), pp.2668-2674
Main Authors: Miyamichi-Yamamoto, Saori, Fukumoto, Yoshihiro, Sugimura, Koichiro, Ishii, Tomonori, Satoh, Kimio, Miura, Yutaka, Tatebe, Shunsuke, Nochioka, Kotaro, Aoki, Tatsuo, Do.e, Zhulanqiqige, Shimokawa, Hiroaki
Format: Article
Language:English
Subjects:
Adult
Aged
Connective tissue disease
Cyclophosphamide - administration & dosage
Female
Glucocorticoids - administration & dosage
Humans
Hypertension, Pulmonary - diagnosis
Hypertension, Pulmonary - drug therapy
Hypertension, Pulmonary - physiopathology
Immunosuppression
Immunosuppressive Agents - administration & dosage
Immunosuppressive therapy
Male
Middle Aged
Prognosis
Pulmonary arterial pressure
Pulmonary vascular resistance
Retrospective Studies
Vascular Resistance - drug effects
Vasodilator Agents - administration & dosage
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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. Methods and 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±11mmHg, P
ISSN:1346-9843
1347-4820
DOI:10.1253/circj.CJ-11-0473