CT findings in bronchiolitis obliterans organizing pneumonia (BOOP) with radiographic, clinical, and histologic correlation.

The CT features of 12 patients with bronchiolitis obliterans organizing pneumonia (BOOP) were reviewed and correlated with clinical history, histologic specimens, and chest radiography. From our series, a spectrum of CT findings of parenchymal lung involvement in BOOP emerged. Focal nodular or mass-like opacities were found in 42% (5 of 12). Areas of consolidation resembling pneumonia were seen in 33% (4 of 12). Peripheral subpleural reticular opacities were identified in 25% (3 of 12). Patchy ground glass infiltrates were seen in 8% (1 of 12). One patient demonstrated a mixed pattern consisting of nodular opacities and areas of pneumonic consolidation. In 4 of the 5 cases demonstrating the nodular form of BOOP either a feeding vessel or bronchus sign could be identified. This feature consisted of a pulmonary vessel leading to a nodular opacity or an air bronchogram entering into a nodular opacity. Correlation of the CT findings of BOOP with histologic specimens showed nodular opacities and areas of consolidation to be associated with classic pathologic features of BOOP including bronchiolar plugs of granulation tissue and surrounding organizing pneumonia. Cases demonstrating peripheral subpleural reticular opacities showed, in addition to pathologic evidence of BOOP, other features such as interstitial disease and fibrosis.

Primary pulmonary botryomycosis. A manifestation of chronic granulomatous disease.

A 19-year-old woman with a childhood history of cavitating left upper lobe pneumonia presented with persistent weight loss, fever, cough and roentgenographic evidence of right upper lobe pneumonia resistant to antibiotic therapy. An open lung biopsy led to the diagnosis of botryomycosis. Neutrophil function studies including flow cytometric evaluation of oxidative burst, bacterial killing and evaluation of neutrophil cytosolic proteins required for oxidase activation were consistent with chronic granulomatous disease. This is the first case report of primary pulmonary botryomycosis as a clinical manifestation of CGD. Other recent cases of immunodeficiency states associated with botryomycosis are reviewed.

Sputum cytopathology: use and potential in monitoring the workplace environment by screening for biological effects of exposure.

Sputum cytopathologic monitoring detects squamous cell lung cancers at an extremely early stage (x-ray negative). It holds further potential for preventing disease by detecting epithelial alterations which reflect environmental hazards. The addition of sputum cytology screening to screening by chest x-ray film does not significantly reduce mortality from all types of lung cancer, but preliminary analysis of Johns Hopkins Lung Project data suggests that mortality from squamous cell carcinoma is reduced. Quantitative automated cytopathology systems and biochemical/immunological cell markers enhance understanding of these precursors and offer great promise for increasing capacity, accuracy, and usefulness in cytopathology screening of workers. Cytological specimens collected over years of screening workers considered at risk may be important to eventually understanding development and prevention of major occupational diseases.

Early lung cancer detection: results of the initial (prevalence) radiologic and cytologic screening in the Johns Hopkins study.

The Johns Hopkins Lung Project was designed to determine whether the addition of cytologic screening to the radiographic screening of high-risk volunteers could enhance the early detection of asymptomatic lung cancer and whether early therapeutic intervention in detected cases could significantly reduce the mortality from this disease. Male volunteers, 45 yr of age and older, who smoked at least 1 pack of cigarettes per day were recruited from the Baltimore metropolitan area. All of the 10,387 acceptable high-risk volunteers received annual chest radiographic screening. By random assignment, one half received cytologic examination of induced sputum in addition to the roentgenogram. This report describes the results of the initial screening. Compared with usual methods of clinical diagnosis, screening by both roentgenography and cytology identified a greater proportion of the lung cancer cases at an earlier stage. Screening by sputum cytology was found to improve the detection only of squamous cell carcinoma. In the dual-screen group, sputum cytology accounted for 28% of the detected cases, and resulted in 39% additional detection of lung cancer over that achieved by roentgenography. There was no corresponding decrease in prevalence. Lung cancers detected by cytology alone were found at very early stages. Although there has been an increase in average survival, much of this increase, if not all, may have resulted from lead-time and sampling bias.

Lung cancer mortality in males screened by chest X-ray and cytologic sputum examination: a preliminary report.

Observations from the continuing JHLP lung cancer screening study provide no clear evidence that mass screening by chest X-ray and sputum cytology, plus prompt appropriate treatment, reduces mortality du to lung cancer. No reduction in lung cancer deaths is observed in the JHLP when compared with the number of lung cancer deaths expected from the experience of two unscreened male populations of the same age and smoking status serving as population studies. Possible reasons for the lack of demonstrable decrease in mortality, even though "early" cases have been detected, include: (1) an insufficient passage of time for slowly growing cancers to become apparent in the control group, (2) ineffective therapy, and (3) the multifocal nature of pulmonary neoplasms. Additional study will be required to provide a definitive answer regarding the benefit, if any, of lung cancer screening.

Risk factors in chronic obstructive pulmonary disease (COPD).

In a genetic-epidemiologic study of chronic obstructive pulmonary disease (COPD) observations adjusted for age, sex, race, and smoking indicate certain factors to be associated with increased pulmonary function aberrancy, and suggest that they are risk factors for COPD. These presumptive "risk factors" include not only cigarette smoking, but also alpha1-antitrypsin (Pi system) variation, one or more other familial components, low socioeconomic status (SES), and, in whites, ABO blood type (either absence of "B" or presence of "A").

A comparison of pulmonary function in male smokers and nonsmokers.

Results of certain tests of pulmonary function, including a questionnaire, single-breath N2 test of closing capacity, forced expiration, and diffusing capacity were significantly different in groups of male smokers and nonsmokers. The influence of age on these smoking-related changes of pulmonary function was evaluated. The analyses indicated that (1) some tests including number of symptoms; closing capacity, i.e., closing volume plus residual volume as a percentage of total lung capacity; residual volume as a percentage of total lung capacity; Phase III of the single-breath N2 test, and steady-state diffusing capacity (ml of CO/mm Hg - min) revealed significant differences between adjusted mean smoker and nonsmoker values but did not reveal differences associated with age. (2) Tests of forced expiration (1-sec forced expiratory volume/vital capapity, reciprocal of the maximal mid-expiratory flow, maximal flow at 50 per cent of vital capacity; and moments) however, revealed differences between smoker and nonsmoker means )adjusted and unadjusted), as well as increasing smoker-nonsmoker differences with increasing age. It is suggested that the first group of tests probably measured an all-or-none response that occurred with the onset of smoking and was not affected by duration of smoking. The second group of tests probably measured the effects of continued smoking and indicated increasing abnormality associated with longer exposure (years of smoking). Test showing age-related differences between smokers and nonsmokers may reflect cummulative, irreversible changes in pulmonary function to a greater extent than test that do not.

Lung volumes and compliance in myasthenia gravis and reversible airway obstruction.

A patient with myasthenia gravis, reversible airway obstruction, and a vertical chest wall pressure-volume curve was shown to inflate his lungs to 12 liter during the night. During the day, either spontaneously or after isoproterenol, his functional residual capacity decreased by more than 2 liter. The large lung volume changes occurred without a change in slope of the static pressure-volume curve of the lung above functional residual capacity. The changes in the position of this curve might be due to the the very slow filling and emptying of bullae or to unexplained alterations in the length-tension characteristics of the alveoli. The vertical pressure-volume curve of the chest wall suggested that, unlike other patients who have normal or decreased chest wall compliance, this patient's chest wal was not a factor that limited ventilation at high lung volumes.

Extended experience in the long-term corticosteroid treatment of pulmonary sarcoidosis.

A 15-yr experience with symptomatic pulmonary parenchymal sarcoidosis treated with corticosteroids has included 250 patients. Within the past 5 yr 192 patients have been actively studied and these are reported in detail. Varied clinical observations are described. Serial measurements of vital capacity and steady-state carbon monoxide diffusing capacity were performed. All of these patients were sufficiently symptomatic and incapacitated to require treatment. Ninety-one percent were Black, 72% were female, and 72% were in the third and fourth decades. In response to treatment there was overall clinical improvement and an increase in vital capacity in 150 (83%) of 180 patients, although only a temporary increase in 12 of these patients. By contrast, only 82 (46%) showed an increase in carbon monoxide steady-state diffusing capacity and only temporarily in 19 patients. Changes in vital capacity correlate well with changes in symptoms and the radiographic appearance and are of great assistance in assessing the clinical course and need for continued treatment. The diffusing capacity is more difficult to interpret. Continued treatment was required in 74 patients (48%) because of recurrent clinical relapses as treatment was withdrawn. Such relapses were observed in 70% of the patients. Improvement and discontinuation of treatment was achieved in 66 patients (42%). In the 44 in whom there was no observed relapse, 34 were treated for less than 2.5 yr. In the total group, longer periods of treatment, 2.5-10 yr or more, were required in 104 patients (54%). In this series of 192 patients there were 7 deaths, with 22 deaths in the total 15-yr series of 250 patients. Of these, 18 were directly attributed to the sarcoidosis. Clinical improvement coincident with corticosteroid treatment and the frequency of relapse as treatment is withdrawn strongly support the benefits of corticosteroid treatment for symptomatic pulmonary sarcoidosis. When relapses occur and recur, retreatment is necessary and often long-term maintenance doses of prednisone (10-15 mg daily) are required for many years. The relatively infrequent problems related to this treatment are greatly exceeded by the clinical benefits.

A genetic-epidemiologic study of chronic obstructive pulmonary disease. I. Study design and preliminary observations.

Although certain environmental agents (e.g., cigarette smoking) are known to be causally related to chronic obstructive pulmonary disease (COPD), differential response to their deleterious effects suggests the importance of constitutional (host) factors. The voluminous literature on the familial occurrence of COPD as well as the association between genetically determined serum alpha 1-antitrypsin (alpha 1-at) deficiency and COPD, however, reveals many aspects yet to be clarified. Studies of alpha 1-at indicate that neither its nature nor its relationship to COPD is simple. Moreover, other familial factors are likely involved. To obtain a better understanding of the etiology and pathogenesis of COPD, both genetic and environmental factors are being examined in a multifaceted investigation. The preliminary observations are summarized. COPD patients have a higher frequency of Pi variant phenotypes than those without lung disease. Among other subjects, both cigarette smoking and Pi variant phenotypes are associated with increased pulmonary function abnormality. Finally, there is familial aggregation of pulmonary impairment that cannot be explained entirely by Pi type or smoking.

Chronic obstructive pulmonary disease and alpha-1-antitrypsin (Pi) variation: a family study.

A kindred of a white male proband with alpha1-antitrypsin deficiency, ZZ phenotype, and a severe obstructive ventilatory defect secondary to pulmonary emphysema was studied with regard to alpha1-antitrypsin phenotype, serum trypsin inhibitory capacity (T.I.C.), and pulmonary function. T.I.C.'s and alpha1-antitrypsin phenotypes were consistent with autosomal codominant inheritance. While MZ relatives as well as the proband had pulmonary function abnormalities, the pattern varied within the kindred and abnormalities were observed in some MM relatives also.

Measurement of regional lung function in the early detection of chronic obstructive pulmonary disease.

Radioisotopic regional lung function measurements using both 99mTc-labeled albumin microspheres and inhaled 133Xe were compared to measurements of total lung function in a population of 30 participants in an epidemiological study of the causative factors of obstructive pulmonary disease. Five of the 8 asymptomatic subjects who had no evidence of obstruction by the tests of total function had abnormal regional lung function measurements. The closing volume was abnormal in three of these five, suggesting the presence of small airways disease. Regional lung function was abnormal in all subjects who were symptomatic, who had a FEV1/FVC less than 75%, or who had an elevated closing volume or residual volume. The data indicate that the measurement of regional lung function may be a highly sensitive test for the early diagnosis of chronic obstructive pulmonary disease.