Pulmonary benign metastasising leiomyoma: response to treatment with goserelin.

Benign metastasising leiomyoma is a rare disease occurring predominantly in women of childbearing age and is hormonally influenced. The response of the disease to the luteinising hormone releasing hormone analogue goserelin is reported.

A double-blind, placebo-controlled comparison of the efficacy of standard and individually titrated doses of theophylline in patients with chronic asthma.

Forty adult patients with chronic asthma completed a 3-month double-blind crossover study to compare the effect of sustained-release theophylline given both as a fixed 300 mg twice daily dose (standard) and an individually titrated dose (titrated) with placebo. Theophylline was given in addition to other usual therapy, inhaled bronchodilators, inhaled steroids and, in 12 patients, oral steroids. The 3-month period was preceded by a run-in phase to determine the dose of theophylline which each subject required to achieve peak serum levels of 12-20 mg/litre and trough levels of 8-12 mg/litre. Doses ranged from 300 mg to 700 mg twice daily. Twenty-one patients needed more than the standard dose to achieve satisfactory serum levels. Patients recorded daily peak flow rates and symptom scores and were seen at monthly intervals to measure lung function, check serum theophylline levels and change treatments, which were given in random order. FEV1 was significantly higher for the whole group after standard (2.11 litres) and titrated (2.15 litres) theophylline therapy than after placebo (1.89 litres), as was FVC, but in the large subgroup whose titrated dose was greater than the standard dose, the FEV1 only improved with the titrated dose. Peak flow measurements at home showed the same pattern. Patients taking oral steroids appeared to derive less benefit from theophylline than others. It is concluded that theophylline can usefully be added as a third-line drug in chronic asthma, but that since half the patients are likely only to benefit from a dose greater than 300 mg twice daily, while the other half may have high serum levels above this dose, it is essential to measure serum levels in order to use the drug effectively and safely.

Red cell folate levels, food antibodies and reticulin antibodies in farmer's lung--is there an association with coeliac disease?

Indirect evidence of enteropathy has been sought in a group of 57 subjects with farmer's lung (FL) by clinical questionnaire, estimation of red cell folate levels, the presence of precipitating antibodies to various food antigens and detection of reticulin antibodies. One subject was found who had villous atrophy and responded clinically to gluten withdrawal. There were two subjects with low red cell folate levels and multiple food antibodies, and one subject with reticulin antibodies and multiple food antibodies. Enteropathy is infrequent in subjects diagnosed clinically as having farmer's lung but seems to be more common than would be expected by chance. The mechanism has yet to be elucidated.

Cryptogenic obliterative bronchiolitis in adults.

Patients referred for assessment of severe chronic airflow obstruction over a three-year period were reviewed, and when all smokers and ex-smokers, those with asthma, chronic bronchitis, emphysema, and other specific pulmonary diagnoses were excluded 10 patients remained. Their clinical, lung function, and bronchographic features were consistent with obliterative bronchiolitis. Nine were women, five had rheumatoid arthritis, and five had survived for more than 10 years after first symptoms. Obliterative bronchiolitis has not previously been considered as a cause of chronic airflow obstruction but the distinctive features suggest that it is a true disease entity.

Empyema presenting over thirty years after pulmonary tuberculosis.

Four patients are described who presented with empyema between 32 and 53 years after pulmonary tuberculosis had been treated by artificial pneumothorax (A.P.) without chemotherapy. Diagnostic problems included non-specific radiographic features and failure to find a causative organism. Empyema should be considered when patients with old pulmonary tuberculosis deteriorate and there are non-specific radiographic changes. Antituberculosis chemotherapy, or even decortication, may be indicated if a pyogenic organism cannot be found.

A study of immunoglobulin G subclasses in patients with farmer's lung.

A quantitative estimate of the IgG subclasses in patients with farmer's lung has revealed significantly higher IgG3 levels in patients with this condition compared to an age-matched group of control farmers similarly exposed to Micropolyspora faeni (P less than 0.01) and a group of normal blood bank donors (P less than 0.001). The IgG1 and total IgG levels were significantly higher in both groups of farmers compared to blood bank controls (P less than 0.001). The possible significance of these observations is discussed in relation to susceptibility to the disease and its pathogenesis.

Association of HLA B8 with spontaneous resolution in sarcoidosis.

HLA may influence the natural history of some diseases. HLA frequencies have been compared in 164 healthy control subjects, 50 patients with sarcoid lung fibrosis, and 37 patients with sarcoidosis that resolved spontaneously. B8 was increased significantly in the resolved group compared to both healthy control subjects (p less than 0.001) and the fibrotic group (p less than 0.01). The results support the findings of other investigators, and are consistent with the hypothesis that inherited host factors, perhaps related to immune response, influence the clinical expression of sarcoidosis.

Different distributions of kinetically apparent molecular varieties of alpha 2-macroglobulin in health and in patients with cryptogenic fibrosing alveolitis.

1. Proteinase inhibitors have been studied in whole serum by using a kinetic method that avoids potentially damaging protein separation procedures. 2. The alpha 2-macroglobulin of an individual can be allocated unambiguously into one of seven categories according to the binding of trypsin to inhibitor in two kinetically apparent binding modes (beta- and alpha-modes). 3. The distribution of alpha 2-macroglobulin beta:alpha ratios in a healthy adult population is defined, and shown to be independent of sex and age. 4. The distribution of beta:alpha ratios in a group of patients with cryptogenic fibrosing alveolitis was found to be significantly different (P less than 0.005) from the normal distribution. 5. Changes in the beta:alpha ratio were noted in five of six patients with cryptogenic fibrosing alveolitis after treatment, but on no occasion when two healthy subjects were assessed a total of nine times. 6. The molecular interpretation and the possible importance of altered proteinase inhibition in inflammation and fibrosis are discussed.

Raised enzyme markers of chronic inflammation in asymptomatic farmers' lung.

Subclinical inflammatory activity may be one of the factors which influences the variable natural history of farmers' lung. Serum lysozyme (LYS) and angiotensin converting enzyme (ACE) have been measured in 52 farmers with a previous history of farmers' lung and in 51 healthy control farmers. The group with farmers' lung assessed during the winter, although having had no recent acute symptoms, had significantly higher levels of LYS and ACE compared to both healthy control farmers seen in winter and farmers' lung subjects seen in summer. There was a mild but significant negative correlation between LYS and farm size in the farmers' lung subjects assessed in winter. The results suggest that active inflammation may be present in subjects with farmers' lung in the absence of acute symptoms during the season when dust exposure occurs as the result of feeding cattle.

Echocardiographic method for the estimation of pulmonary artery pressure in chronic lung disease.

The prognostic implications of pulmonary hypertension in chronic lung disease, and the difficulty in establishing its severity without cardiac catheterisation, indicate the need for a reliable non-invasive method of assessing the pulmonary artery pressure. It is likely that the time taken by the right ventricle to generate a sufficiently high pressure to open the pulmonary valve will increase progressively as the pulmonary arterial diastolic pressure rises. Therefore, the time interval between closure of the tricuspid valve and opening of the pulmonary valve has been obtained by high-speed echocardiographic recordings of the tricuspid and pulmonary valves in a group of 17 patients with chronic lung disease. Each patient underwent right heart catheterisation immediately after the echocardiographic examination so that the pulmonary arterial diastolic pressure could be obtained directly. A range for the group from 15 mmHg (2.0 kPa) to 45 mmHg (6.0 kPa) was observed. The linear correlation between the measured diastolic pressure and time interval from the tricuspid valve closure to pulmonary valve opening was highly significant (r = 0.94, p = less than 0.001) and the scatter was relatively small. It is therefore suggested that this time interval, obtained non-invasively by echocardiography, can be used as an index of the severity of pulmonary hypertension associated with chronic lung disease.

Bronchoalveolar lavage fluid cell counts in cryptogenic fibrosing alveolitis and their relation to therapy.

Bronchoalveolar lavage was used to sample inflammatory cells from the lungs of 51 patients with cryptogenic fibrosing alveolitis (CFA) (24 smokers, 12 ex-smokers, and 15 non-smokers). The smokers with CFA have been compared with 15 smoking control subjects in whom there was no radiographic abnormality or clinical evidence of chronic bronchitis. Significantly lower volumes of lavage fluid were recovered from the smokers with CFA (p < 0.001) and the fluid contained lower percentages of macrophages (p < 0.01), reflecting increased percentages of eosinophils (p < 0.001) and neutrophils (p < 0.01). Similar changes were seen in the ex-smokers and non-smokers. There was also an increase in the percentages of lymphocytes when the whole group of CFA patients was compared with the control subjects (p less than or equal to 0.05). No significant differences were found when patients with "lone" CFA were compared with those having associated systemic disease. The only feature distinguishing smokers from non-smokers with CFA was the presence of pigmented cytoplasmic inclusions in the macrophages from the smokers (p < 0.001). However, there were lower numbers of pigmented macrophages in the smoking CFA patients by comparison with the control subjects suggesting either a change in phagocytic capacity or turnover rate in this disease. Profiles of differential cell counts in individual patients showed that increases of eosinophils over 3% or neutrophils over 4% or both with lymphocyte counts of less than 11% related to a poor clinical response to corticosteroids, but lymphocyte percentages greater than 11% related to improvement (p < 0.05).

Pleural effusions.

Pleural effusions develop following changes in capillary permeability, capillary hydrostatic pressure, plasma colloid osmotic pressure, or lymphatic drainage. Generalized fluid retention or a transudate suggests a systemic cause while an exudate suggests a local cause. The diagnosis can usually be established by clinical assessment, chest radiography, analysis of pleural fluid, pleural biopsy, and appropriate special investigations. When no cause can be found, particular care should be taken to exclude secondary carcinoma, tuberculosis, pulmonary infarction, and mesothelioma; the patient should be followed-up. Symptomatic malignant effusions may be managed initially by basal intercostal tube drainage alone. For recurrences, local instillation first of tetracycline and then of mustine may be tried. Pleurectomy should be considered if the prognosis is otherwise good.