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The role of interleukin-10 gene expression in alveolar macrophages in the pathogenesis of interstitial lung disease (ILD) was investigated. Patients with idiopathic pulmonary fibrosis (IPF) and bronchiolitis obliterans with organizing pneumonia (BOOP) along with normal subjects were evaluated to compare the level of expression of tumor necrosis factor (TNF)-alpha and interleukin-10 in alveolar macrophages. Results showed that alveolar macrophages in patients with IPF and BOOP experienced an increase in interleukin-10 mRNA expression compared with healthy subjects.

Idiopathic pulmonary arterial hypertension (IPAH) is pathogenetically related to low levels of the vasodilator nitric oxide (NO). Because NO regulates cellular respiration and mitochondrial biogenesis, we hypothesized that abnormalities of bioenergetics may be present in IPAH. Evaluation of pulmonary artery endothelial cells from IPAH and control lungs in vitro revealed that oxygen consumption of IPAH cells was decreased, especially in state 3 respiration with substrates glutamate-malate or succinate, and this decrease paralleled reduction in Complex IV activity and IPAH cellular NO synthesis. IPAH pulmonary artery endothelial cells had decreased mitochondrial dehydrogenase activity and lowered mitochondrial numbers per cell and mitochondrial DNA content, all of which increased after exposure to NO donors. Although IPAH/pulmonary artery endothelial cells' ATP content was similar to control under normoxia, cellular ATP did not change significantly in IPAH cells under hypoxia, whereas ATP decreased 35% in control cells, identifying a greater dependence on cellular respiration for energy in control cells. Evidence that glucose metabolism was subserving the primary role for energy requirements of IPAH cells was provided by the ≈3-foId greater glycolytic rate of IPAH cells. Positron emission tomography scan with [18F]fluoro-deoxy-D-glucose performed on IPAH patients and healthy controls revealed significantly higher uptake in IPAH lungs as compared with controls, confirming that the glycolytic rate was increased in vivo. Thus, there are substantial changes in bioenergetics of IPAH endothelial cells, which may have consequences for pulmonary hypertensive responses and potentially in development of novel imaging modalities for diagnosis and evaluation of treatment. [ABSTRACT FROM AUTHOR]

Deals with a study which evaluated the expression of nitric oxide synthase (NOS) and the factors that regulate NOS activity in patients with pulmonary arterial hypertension (PAH). Clinical characteristics of the study population; Level of serum arginine on patients with PAH; Schematic diagram of impaired biosynthesis of NO in adult primary pulmonary hypertension.

The diagnosis of pulmonary hypertension (PH) relies on a high index of suspicion. In patients with symptoms or chest radiographic findings suggestive of PH, a detailed history and physical examination followed by early assessment with a transthoracic echocardiogram, ventilation-perfusion scanning, chest computed tomography, pulmonary function testing, and nocturnal oximetry screening can provide valuable information about etiology and severity. Right heart catheterization should follow in patients who are symptomatic or who demonstrate moderate to severe PH by echocardiography and are candidates for treatment. Patients at risk for developing PH should undergo serial echocardiography and pulmonary function testing to assess for disease development and progression. Genetic testing is not currently recommended in the routine evaluation of patients with a diagnosis of primary pulmonary hypertension.