Chest
Original ResearchImmunosuppressive Therapy in Connective Tissue Diseases-Associated Pulmonary Arterial Hypertension
Section snippets
Patients
Between February 1991 and January 2001, 112 patients with connective tissue disease were referred to our institute for PAH management (systemic scleroderma, n = 61; SLE, n = 24; MCTD, n = 17; polymyositis, n = 2; Sjögren syndrome, n = 4; rheumatoid arthritis, n = 4). Among them, 28 patients (25%) were not previously treated with either PAH-specific therapies (IV, subcutaneous, inhaled, or oral prostacyclin derivatives, endothelin receptor antagonists, or phosphodiesterase type 5 inhibitors) or
Baseline Characteristics
Individual baseline demographic and hemodynamic parameters are summarized in Table 1. Most patients were female (82%), with a mean age of 40 ± 17 years. PAH was severe (Table 1), with a mean 6-min walk distance of 256 ± 109 m, and 75% of the patients were in NYHA functional class III (57%) or IV (18%).
Response to Immunosuppressive Therapy
Eight of 28 patients (29%) were responders to immunosuppressive therapy, as defined above. Five of eight responders had SLE (62%), and the remaining three responders had MCTD (38%). Clinical and
Discussion
Pulmonary hypertension is a major complication of connective tissue diseases. In some patients, pulmonary hypertension is due to either significant left-heart disease or chronic respiratory failure with hypoxemia, requiring specific management.2324 However, in many cases, the degree of pulmonary hypertension is not due to pulmonary fibrosis or left-heart disease and corresponds to chronic obstruction of small pulmonary arteries characteristic of PAH.2526 Advances in the understanding of the
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Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).
This study was supported by grants from Universite Paris-Sud, Legs Poix, and Institut des Maladies Rares.