Intrathoracic lymphangiomatosis mimicking lymphangioleiomyomatosis in a young woman.

In this report, we describe a case of lymphangiomatosis that mimicked lymphangioleiomyomatosis in a 20-year-old woman. Lung biopsy specimens showed proliferation of anastomosing lymphatic channels in the visceral pleura and dilated peribronchiolar and septal lymphatic channels. During 8 years of follow-up, this process behaved like lymphangioleiomyomatosis with reticulonodular infiltrates and worsening obstructive and restrictive changes evident on pulmonary function tests.

Silo-filler's disease.

Between 1955 and 1987, 17 patients were examined at the Mayo Clinic shortly after exposure to silo gas. All exposures had occurred in conventional top-unloading silos. Acute lung injury occurred in 11 patients, 1 of whom died; early diffuse alveolar damage with hyaline membranes and hemorrhagic pulmonary edema and acute edema of the airways were found at autopsy. In one patient, hypoxemia and transient obstruction of the airways developed, but no pulmonary infiltrates were noted. One patient had symptoms for 5 weeks and diffuse confluent pulmonary infiltrates; many years later, chronic obstructive pulmonary disease developed (he had, however, been a heavy smoker before exposure). Bronchiolitis obliterans was not observed in the other patients, probably because of less severe exposure or early corticosteroid therapy. Prophylactic corticosteroid therapy is advised for workers who have been exposed to silo gas. The management of patients with established acute lung injury is reviewed. Previously unreported patterns of exposure to silo gas in conventional silos are described, and recommendations for avoiding exposure are suggested.

Long-term follow-up of persons attending a community-based smoking-cessation program.

The long-term effectiveness of a smoking-cessation program based on education, behavior modification, and group support was evaluated. Because of the availability of long-term follow-up data, the natural history of cessation of smoking in persons who had attended a smoking-cessation program could be studied. At 1 year, the permanent rate of cessation of smoking was 22%, and permanent cessation continued to occur at least through 6 years of follow-up. Factors related to a higher rate of cessation of smoking were a white-collar occupation, having previously stopped smoking for more than 1 month or more than 1 year, being male, and wanting to stop smoking because of health concerns. In the absence of an almost complete return of questionnaires, sole reliance on mailed self-reports would have substantially overestimated the smoking-cessation rates. Although smoking-cessation programs alone involve relatively few smokers, analysis of the methods used in programs such as the one described continues to be important for the development of successful efforts to help people stop smoking.

Respiratory tract symptoms as a clue to giant cell arteritis.

Although many manifestations of giant cell arteries are increasingly recognized, little attention has been paid to respiratory symptoms associated with this disorder. We report the cases of 16 patients with giant cell arteritis who had prominent symptoms related to the respiratory tract including cough, sore throat, and hoarseness. These symptoms were the initial finding in 10 patients and obscured the diagnosis in some instances, but resolved quickly when corticosteroids were given. It is estimated that 9% of patients with giant cell arteritis have prominent respiratory tract symptoms, which are the initial manifestation in 4%. This disorder should be considered in an older patient with a new cough or throat pain without obvious cause.

Influence of height on the risk of spontaneous pneumothorax.

The risk of primary spontaneous pneumothorax was found to be greatest among persons 25 to 34 years old of each sex and greater for men than women, although a gradient of risk with increasing height was found which seemed to explain much of the male predominance in this condition. Minor physical anomalies were relatively common among these patients, but no specific clinical syndromes could be identified. For secondary spontaneous pneumothorax, the risk increased with age and was greater for men, although this may simply have reflected an increased frequency of underlying chronic pulmonary disease. The gradient of risk with height was much less pronounced for secondary spontaneous pneumothorax. Most of the patients in both pneumothorax groups smoked.

The outcome of patients with pleural effusion of indeterminate cause at thoracotomy.

In 51 patients, the cause for pleural effusion remained indeterminate immediately after thoracotomy. Thirty-one (60.8%) had no recurrence of the effusion, and no cause became apparent during a follow-up period of from 1 1/2 to 15 years. Two patients (3.9%) died relatively soon after thoracotomy, but death was not clearly related to the pleural effusion. In 18 patients (35.3%), the cause of the effusion became apparent from 12 days to 6 years after thoracotomy. In 13 of these 18 patients, malignancy (6 patients with lymphoma, 4 with malignant pleural mesothelioma, and 3 with other malignancy) was ultimately diagnosed. In 3 of the 18 patients, the ultimate diagnosis was a collagen-vascular disease, and 1 patient each had the yellow-nail syndrome and mitral stenosis.

Chronic obstructive pulmonary disease: a community-oriented program including professional education and screening by a voluntary health agency.

A community program for chronic obstructive pulmonary disease that included detection by screening, professional education, community involvement, and evaluation of results is described. Key figures in the success of this program were senior medical students. In the first community, in which no professional education was offered, 45.3% of persons screened who had abnormal test results came away from their physicians understanding that they did not have a significant health problem. In subsequent communities, in which professional education was a part of the program, this percentage decreased steadily to approximately 11%. Consistently, 25% of the persons screened who had abnormal results did not consult a physician. Twenty-one % of the participants with abnormal results of screening and no previous knowledge of pulmonary disease stopped smoking in the ensuing 2 to 3 yr, whereas only 11.7% of the control group of persons with normal screening results did so.

Incidence of spontaneous pneumothorax in Olmsted County, Minnesota: 1950 to 1974.

Between 1950 and 1974, 318 cases of initial pneumothorax were diagnosed among the residents of Olmsted County, Minn. Seventy-five cases were due to trauma, and 102 were iatrogenic. One hundred forty-one cases were spontaneous in onset, of which 77 were primary and 64 secondary to a specific underlying pulmonary disease. The age-adjusted incidence of primary spontaneous pneumothorax was 7.4/100,000/yr for males and 1.2/100,000/yr for females. For secondary spontaneous pneumothorax, the incidence was 6.3 and 2.0/100,000/yr for males and females, respectively. The ratio of male-to-female incidence was 6.2:1 for primary and 3.2:1 for secondary spontaneous pneumothorax. The differences between the incidence rates for primary and secondary pneumothorax by sex or in total were not significantly different.

Desmoid tumors of the chest wall.

Twenty-six cases of desmoid tumors of the wall of the chest were reviewed. These are rare lesions, which nonetheless should be considered in the differential diagnosis of all tumors of the chest wall. Although the lesion is most often palpable, several of the tumors were detectable only by means of an x-ray film of the thorax. Definitive diagnosis could not be made clinically but was easily established by pathologic examination of widely excised tumor. Like all desmoid tumors, these were locally aggressive in their growth but nonmetastatic and generally carried a good prognosis. Ideally, treatment consisted of wide local excision, but if there was encroachment of the tumor on vital structures, excision with suboptimal tumor-free margins was required. Even in the latter instance, tumefaction could be well controlled for long periods. Recurrences after an initial attempted removal were common, but most of these responded to reexcision.

Pulmonary tumorlets associated with Cushing's syndrome.

Tumorlets of the lung generally have been considered benign nests of cells, usually found with bronchiectasis or fibrosis. We saw a patient with Cushing's syndrome whose tumorlets ultimately became roentgenographically visible as multiple small nodules throughout both lungs and metastasized to the mediastinum. The plasma corticotropin concentration was increased, as were the concentrations of corticotropin-like peptides in the tumor tissue. This experience lends support to the concept that some tumorlets arise from Kulchitsky's cells in the epithelium of peripheral bronchioles, as do carcinoid tumors, and have the same peptide-producing potential. The possibility of such tumors should be considered in patients with Cushing's syndrome who have no initial roentgenographic abnormality.

Pleural fluid complement, complement conversion, and immune complexes in immunologic and nonimmunologic diseases.

Forty-four pleural fluids and 41 blood specimens from patients with various diseases were examined for concentration of whole complement, C4, C3, conversion products of C3 and C3PA, and immune complexes. C3 conversion was found in all eight pleural fluids from patients with rheumatoid arthritis, five of seven with lupus erythematosus, two of six with congestive heart failure, and nine of 23 with malignant diseases. Conversion of C3PA correlated closely with C3 conversion and both were significantly inversely related to whole complement, C4, and C3. Concentration of immune complexes was highest in patients with rheumatoid arthritis. Pleural fluid immune complex concentrations correlated positively with conversion of C3 and C3PA. These findings suggest that the reduced levels of pleural fluid complement in rheumatoid arthritis and lupus erythematosus may be secondary to complement conversion by immune complexes.

Host factors in chronic obstructive pulmonary disease in an upper Midwest rural community. Design, case selection, and clinical characteristics in a matched-pair study.

A series of 111 index subjects with chronic obstructive pulmonary disease (COPD) who had forced expiratory volume in 1 second (FEV1) of 70% or less of that predicted were matched on the basis of age, sex, occupation, and smoking history with control subjects who had an FEV1 of 85% or more of that predicted. Index and control subjects with seasonal or reversible airway disease were excluded. Men outnumbered women by a ratio of 4.5 to 1. Thirty-five percent of the women and 2% of the men were nonsmokers (0 pack-years). There were three PiZ phenotypes in the index group (two nonsmokers) and none in the controls. PiMZ phenotypes in the index group outnumbered those in the controls by 8 to 5. Host factors that might be important in these closely matched pairs were sought by history, physical examination, and a large battery of laboratory tests. A standard respiratory questionnaire revealed the anticipated significantly higher frequency of cough, phlegm, noisy respiration, and all grades of dyspnea in index subjects. Previous lower respiratory tract infections also were more frequent in index subjects than in controls. There were no detectable differences between groups in the frequency of upper airway infections, nasal polyps, sinus surgery, or reported allergy to any substance. If the British Medical Research Council's definition of chronic bronchitis were applied to our study, about two-thirds of our index subjects and almost one-third of our controls would be considered to have chronic bronchitis. Pack-years of smoking were not significantly associated with the amount and duration of cough and expectoration in male or female index subjects or controls. Significant differences between index and control groups on physical examination included the audible forced expiratory flow time over the trachea, the estimated maximal midexpiratory flow, breath sounds, rales, and total excursion of the hemidiaphragms. An endocrine questionnaire and measurement of blood sex hormones did not give any clues as to the propensity of males to develop COPD. Women with airway obstruction similar to that of men had histories of significantly fewer pack-years than did the men, and there was a much larger proportion of women who never smoked. Further studies, specifically on genetic and immunologic characteristics, are under way to identify potential host factors.