Bronchiectasis is characterized by chronic inflammation in one or more bronchi, but the extent of inflammation is difficult to monitor. The concentration of nitric oxide (NO) in exhaled air is increased in asthmatic patients, possibly as a result of the chronic inflammatory process. We have measured exhaled NO in patients with documented bronchiectasis and investigated whether the concentration of exhaled NO is related to the extent of disease as defined by computed tomography (CT) and lung function. In 20 patients with bronchiectasis who were not taking inhaled steroids, the peak concentration of NO in exhaled air, measured by a modified chemiluminescence analyzer, was significantly elevated (285 +/- 49.0 ppb) as compared with values for 79 normal subjects (89 +/- 2.7 ppb, p < 0.01) and 19 patients with bronchiectasis treated with inhaled steroids (88 +/- 13.4 ppb, p < 0.01). Thin-section CT was used to quantify the extent of bronchiectasis in the 19 patients. There was a significant correlation between the CT score and FEV1 (r = 0.73, p < 0.01). In patients not treated with inhaled steroids there was a significant relationship between CT score and peak exhaled NO (r = 0.81, n = 12, p < 0.02), but this was not the case for patients treated with regular inhaled steroids (n = 7). We conclude that untreated bronchiectasis is associated with an increase in exhaled NO, and that this is correlated with disease severity, whereas patients treated with inhaled steroids have levels of exhaled NO within the normal range.(ABSTRACT TRUNCATED AT 250 WORDS)

Fibrosing alveolitis associated with systemic sclerosis (FASSc) is considered to be histologically and radiologically indistinguishable from lone cryptogenic fibrosing alveolitis (CFA). To date, the natural history of the two diseases has not been compared directly in large groups of patients followed at a single institution. We evaluated the survival of 205 patients with CFA and 68 patients with FASSc. Deaths during the follow-up period were reported in 142 patients with CFA (70%) and in 11 patients with FASSc (16%). Unadjusted survival from the onset of dyspnea was less in CFA than in FASSc (odds ratio, 3.3; p < 0.001); this difference persisted after adjustment for age of onset of dyspnea and smoking history. Survival from presentation was less in CFA (odds ratio approximately 3.3) after adjustment for age of presentation, smoking history, and initial pulmonary function indices. These differences persisted when analysis was confined to patients with histologic confirmation of fibrosing alveolitis and after adjustment for treatment. In patients undergoing computed tomography (CT) of the lungs, survival was less in CFA after adjustment for extent of abnormal lung on CT and CT pattern of disease (odds ratio, 3.9; p < 0.001). These findings indicate that despite their histologic and radiologic similarities, lone CFA and FASSc have different courses.

In most clinical series of patients with cryptogenic fibrosing alveolitis (CFA), disease severity is staged using lung function indices. However, many physiologic indices are measured in routine clinical practice; the choice of variable to evaluate functional severity is contentious. Computed tomography (CT) provides a reproducible means of quantifying the morphologic extent of disease. The aim of this study was to evaluate the functional consequences of smoking-related lung damage in CFA and to identify functional measures best reflecting the extent of fibrosing alveolitis on CT. Sixty-eight patients with CFA were studied. Fourteen patients with emphysema on CT were characterized by relative preservation of FVC and TLC (p < 0.005) and relative depression of DLCO (p < 0.05) and KCO (p < 0.00005). On multivariate analysis, the extent of fibrosing alveolitis and the presence of emphysema were independent determinants of functional impairment; there was no independent relationship between smoking history and functional abnormalities. In patients without emphysema on CT, percent predicted DLCO (r = -0.68), oxygen desaturation on exercise (r = 0.64), and the physiologic component of the clinical-radiographic-physiologic (CRP) score (r = 0.62) correlated much better with the extent of disease on CT than spirometric and plethysmographic volumes. A composite functional index was generated against the extent of disease on CT, using multivariate analysis; comparison with the CRP score suggested that the relationship between morphologic disease extent and the CRP score would be improved by the inclusion of DLCO and by the use of negative weighting for depression of FEV1. These findings indicate that in CFA, the presence of concurrent emphysema on CT has a more profound influence upon functional measures than the smoking history, and underline the importance of both the measurements of DLCO and exercise testing in the assessment of the severity of CFA.

Areas of decreased attenuation and mosaic perfusion are an important ancillary CT finding in hypersensitivity pneumonitis, and obstructive functional abnormalities indicate that this phenomenon is caused by bronchiolitis.

Bronchoscopic therapies to reduce lung volumes in chronic obstructive pulmonary disease are intended to avoid the risks associated with lung volume reduction surgery (LVRS) or to be used in patient groups in whom LVRS is not appropriate. Bronchoscopic lung volume reduction (BLVR) using endobronchial valves to target unilateral lobar occlusion can improve lung function and exercise capacity in patients with emphysema.

Background The relative proportions

Objective. Thin-section computed tomography (CT) provides a sensitive and reproducible method of quantifying the morphologic extent of disease in the clinical management of fibrosing alveolitis associated with systemic sclerosis (FASSc). The aim of this study was to determine which indices of lung function best reflect the extent of disease on CT in FASSc, and to determine the independent influences of smoking history, extent of fibrosing alveolitis, demographic features, and concurrent treatment upon functional impairment in FASSc.Methods. Sixty-four patients with FASSc were studied using CT and static and exercise lung function testing. Statistical relationships were determined by multiple regression analyses.Results. Five patients with overt pulmonary hypertension were characterized by severe impairment in 3 indices of lung function: diffusing capacity for carbon monoxide (DLco), DLco adjusted for alveolar volume (Kco), and arterial partial pressure of oxygen. On multiple regression analysis, the major determinant of functional impairment was the extent of fibrosing alveolitis on CT. A history of smoking was independently associated with preservation of total lung capacity and depression of Kco, but did not otherwise influence functional-morphologic correlations. The percent predicted DLco correlated better with extent of disease on CT (r = -0.70) than did oxygen desaturation on exercise (r = OSS), the physiologic component of the

Niemann-Pick disease should be considered for any patient with unexplained diffuse endogenous lipid pneumonia, even when disease is limited to the lungs and presentation is during adulthood.