Gérard Huchon

Multiple pulmonary arterial aneurysms in Behcet's disease and Hughes-Stovin syndrome.

Four case studies of patients with angiographically documented pulmonary arterial aneurysms are presented. In two cases, Behcets disease was diagnosed; one case corresponded to the syndrome described by Hughes and Stovin, that is, venous thrombosis especially of the vena cava accompanied by singular or multiple pulmonary arterial aneurysms in young patients; and the last case could best be described as an association of the two. Our observations lead us to question the existing notions concerning the relationship between Behcets disease and Hughes-Stovin syndrome-the clinical, angiographic and histologic aspects of the vascular manifestations are comparable. Typically the two diseases run similar courses with death resulting frm the rupture of the aneurysms and massive hemoptysis. These case studies cast certain doubts as to the effectiveness of the corticosteroid treatment usually prescribed. Finally, we suggest that Hughes-Stovin syndrome might be, in fact, a manifestation of Behcets disease.

Assessment of Respiratory Epithelial Permeability by Bronchoalveolar Lavage and Aerosolized 99mT‐DTPA in Patients with Sarcoidosisa

The increased permeability of the respiratory epithelium in many pulmonary diseases has already been described.Id Endothelial permeability may be assessed by several different methods: by using indicator dilution curves and a range of tracer molecules in a single pass through the vascular bed,’ by the measurement of the pulmonary transvascular flux of a radioactive protein using an external scintillation detector,* and by the study of transendothelial transport material after osmotic bolus? These methods have limited application to studies in man, and they measure only the pulmonary endothelial permeability. Other techniques have recently been developed for measuring pulmonary endothelial and epithelial permeability that may be applicable to studies in patients: the determination of albumin flux by the measurement of albumin concentration in alveolar fluid obtained by a bronchoalveolar lavage (BAL),’O-I2 and the determination of the flux of a radioactive solute introduced into respiratory spaces as an aerosol. The transfer of the radioactive solute from alveolar spaces to pulmonary blood circulation may be assessed by taking repeated venous blood samples” or using an external scintillation detector which counts the decay of radioactivity in the lung The purpose of this study was to compare two methods for assessing the

Comparison between theophylline analysis by nephelometric inhibition immunoassay and high performance liquid chromatography.

Rate nephelometric inhibition immunoassay (NIIA) was used to determine 94 serum theophylline concentrations, and these results were compared with those obtained using high performance liquid chromatography (HPLC) as a reference method. The measurements obtained by the nephelometric method were, on the average, 20% higher than those obtained by the chromatographic method (p less than 0.001). The coefficient of variation of the nephelometric method was 12.3%, compared with 3.9% for the chromatographic reference method. In the group of subjects having a serum concentration of caffeine greater than or equal to 0.5 microgram ml-1, the difference in serum theophylline concentration found between NIIA and HPLC correlated with serum caffeine concentration (r = 0.3755, df = 46, p less than 0.01). NIIA theophylline concentration was 8.8 +/- 3.2 micrograms ml-1 in serum from six additional patients receiving theophylline 9.1 +/- 3.4 micrograms ml-1 after adding 10 micrograms caffeine (NS), and 13.1 +/- 3.5 micrograms ml-1 after adding 10 micrograms 1,3-dimethyluric acid (p less than 0.001). We conclude that (a) the results obtained by the NIIA method are more variable and consistently higher than those obtained by the HPLC method and (b) 1,3-dimethyluric acid (a caffeine and theophylline metabolite) is responsible for this overevaluation.

In vitro synthesis of angiotensin-converting enzyme by alveolar macrophages is increased in disseminated sarcoidosis

To determine the responsibility of alveolar macrophage (AM) for the increased concentration of angiotensin-converting enzyme (ACE) in bronchoalveolar fluid of patients with sarcoidosis, we cultured AM recovered by bronchoalveolar lavage of 30 subjects: 9 were normal control subjects (group C), 10 had intrathoracic localized sarcoidosis (group LS), and 11 had disseminated intrathoracic and extrathoracic sarcoidosis (group DS). Cells were cultured for 7 days and synthesis of ACE was evaluated according to the difference between final (ACEqe) and initial (ACEqi) ACE content. In groups C, LS, and DS, ACEqi were, respectively, 3.0 ± 1.7, 3.3 ± 1.9, and 4.8 ± 2.4 U/106 AM, and ACEqe were 6.2 ± 1.7, 6.7 ± 2.5, and 11.3 ± 2.8 U/106 AM. ACEqe was higher than ACEqi in all 3 groups (p < 0.01). When cycloheximide was added to the AM culture, ACEqe did not differ from ACEqi, in opposition to what was observed without cycloheximide (p < 0.002). ACEqi in group LS and DS did not differ from ACEqi in Group C, but ACEqe in Group DS was higher than in Groups C and LS (p < 0.01). We conclude that AM both from normal subjects and from subjects with sarcoidosis contain ACE, cultured AM synthesize ACE, and that a greater amount of ACE is produced when AM are obtained from patients with disseminated sarcoidosis.

Aerosol deposition in the alveolar space

The fate of inhaled drugs depends on the specifications of the aerosol, the drug itself, the conditions of aerosolization, and the presence of lung disease. There are still many points to be elucidated before aerosols can be used more reliably in the treatment of respiratory or nonrespiratory conditions.