title: Peripheral airways obstruction in idiopathic pulmonary artery hypertension (primary).
abstract: The mechanical properties of the lung were studied in ten nonsmokers with idiopathic pulmonary artery hypertension (IPAH) (mean pulmonary artery pressure 65.7 +/- 30 mm Hg). In the routine lung test, residual volume was found to be abnormal (greater than 120 percent of the predicted) in seven patients, and measured airway resistance was normal in eight out of the ten patients. A decreased FEF 75-85 percent, abnormal values for the helium-air flow ratios and increased closing capacities were documented in eight of ten patients in whom lung elastic recoil was normal (six of ten) or increased (four of ten). These features suggest peripheral airways obstruction (PAO) which was also supported by histopathologic findings in three cases (one biopsy and two necropsies). The observed changes in lung compliance could be related to the behavior of the coupling of the air-space and vascular compartments. The etiology of PAO in IPAH patients is not known, but our results indicate that both the peripheral airways and the pulmonary circulation are affected. The knowledge of PAO in IPAH patients could help to better understand the observed V/Q inequality in this entity.
mesh_terms: D000293:Adolescent; D000328:Adult; D000403:Airway Resistance; D001696:Biomechanical Phenomena; D004547:Elastic Tissue / Q000473:pathology; D005540:Forced Expiratory Flow Rates; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000503:physiopathology*; D008170:Lung Compliance; D008173:Lung Diseases, Obstructive / Q000175:diagnosis / Q000503:physiopathology*; D011651:Pulmonary Artery; D011652:Pulmonary Circulation; D012115:Residual Volume