[Goodpasture syndrome: role of an epidemiological factor? Apropos of two cases]. / Le syndrome de Goodpasture: rôle d'un facteur épidémiologique? A propos de deux observations.

Goodpasture's syndrome is a rare pneumorenal syndrome. Although the antigenic target of this auto-immune disorder is now known, its etiology remains debated. We report two cases of Goodpasture's syndrome occurring in similar epidemiologic conditions concerning the moment the disease began, the age and sex of the patients, their place of residence and work and manipulation of chemicals. Thus, a common environmental factor could have been the trigger event of the Goodpasture's syndrome. The epidemiologic features of this disease are reviewed.

[Unilateral agenesis of the pulmonary artery. Diagnosis and therapeutic management]. / Agénésie unilatérale de l'artère pulmonaire. Diagnostic et prise en charge thérapeutique.

A case of agenesia of the right pulmonary artery was observed. The epidemiological and clinical characteristics of this congenital anomaly as well as treatment were reviewed: Surveillance or surgery?

[Round pulmonary lesions after returning from French Guyana. Six cases of american pulmonary histoplasmosis]. / Image ronde pulmonaire au retour de Guyane française. A propos de six cas d'histoplasmose américaine pulmonaire.

From 1989 to 1994, 71 patients were hospitalized for diagnosis of round lung lesions including 49 servicemen under the age of 45 years who had been stationed in tropical areas. In 6 of these servicemen, the diagnosis was pulmonary histoplasmosis at the tertiary stage of histoplasmoma. All had done duty in French Guyana and were negative for human immunodeficiency virus. The subpleural lung opacity was the only lesion in 5 out of 6 patients and was calcified in 4 out of 6 patients. Since skin tests with histoplasmin and serologic testing for histoplasmosis failed to achieve definitive diagnosis, surgical biopsy was performed by conventional thoracotomy in 2 cases and video-assisted thoracic surgery in 4 cases. The specimens obtained confirmed diagnosis of histoplasmosis on mycologic criteria in 3 cases and on a combination of findings including compatible histologic evidence in 3 cases. Treatment consisted in surgical excision of the nodules, for which video-assisted thoracoscopic surgery proved to be an excellent technique because of its simplicity and rapidity.

[Alveolar proteinosis: diagnosis and therapeutic management. Apropos of 2 cases]. / La protéinose alvéolaire: diagnostic et prise en charge thérapeutique. A propos de deux observations.

Two cases of pulmonary alveolar proteinosis are reported. Emphasis is placed on imaging techniques for diagnosis with bronchofibroscopy and bronchoalveolar lavage. Treatment of symptomatic forms is based on classical alveolar lavage with a rigid bronchoscope or currently with the fibroscope.

[Uncommon complication of the use of implantable chambers in oncology]. / Complication inhabituelle de l'utilisation des chambers implantables en oncologie.

The catheter of an implantable chamber migrated into the pulmonary infundibulum in a patient given chemotherapy for bronchogenic cancer. Current management of such cases is to remove the catheter under pulmonary angiography control using a basket system.

[Demonstration of subclinical pulmonary alveolitis in spondylarthropathies]. / Mise en évidence d'une alvéolite pulmonaire infraclinique au cours des spondylarthropathies.

Restrictive ventilatory dysfunction, lowered diffusing capacity, and apical fibrosis have been reported in ankylosing spondylitis. To investigate the pathogenesis of these abnormalities, we studied distal airspace cytology by performing bronchoalveolar lavage in 34 spondyloarthropathy patients (ankylosing spondylitis, n = 16; reactive arthritis, n = 4; axial psoriatic arthritis, n = 2; and undifferentiated spondyloarthropathy with HLA B27-positivity in every case but one, n = 12). Mean age was 32.4 +/- 13.7 years. None of the study patients had apical fibrosis, lower respiratory tract infection, or exposure to airborne pollutants other than tobacco smoke. The control group was composed of nine subjects who had no lung or inflammatory diseases and were not using medications. Significantly higher proportions of lymphocytes were found in bronchoalveolar lavage specimens from patients, as compared with controls. This difference was not influenced by smoking or medication use (non steroidal antiinflammatory drugs, sulfasalazopyridine). Alveolar lymphocytosis was not correlated with laboratory tests for disease activity (erythrocyte sedimentation rate, serum IgA levels) or with the presence of restrictive ventilatory dysfunction. Increases in the proportion of lymphocytes were of similar magnitude in patients with ankylosing spondylitis and in those with other spondyloarthropathies. Absolute total cell counts and relative neutrophil counts were similar in patients and controls. However, among the patients with spondyloarthropathies, those with a disease duration of more than five years had a significantly higher proportion of neutrophils than those with a disease duration of less than five years. These findings demonstrate that spondyloarthropathy patients have subclinical lymphocyte alveolitis. Although of unclear significance, this alveolitis may be related to the development of apical fibrosis in some patients with ankylosing spondylitis.

[Ganglioneuroma of the mediastinum. Apropos of a case]. / Ganglioneurome du médiastin. A propos d'une observation.

The authors report a case of a large ganglioneurinoma located in the mediastinum. The clinical course of this rare benign neurogenic tumour of the posterior mediastinum includes a low risk of medullary compression. The treatment is surgical resection.

[Cytological study of deep lung in spondyloarthritis. Apropos of 34 cases]. / Etude cytologique du poumon profond au cours des spondylarthropathies. A propos de 34 observations.

Bronchoalveolar lavage (BAL) was performed in 34 consecutive patients with spondyloarthropathy (SA) compared with 9 controls. Lymphocytic alveolitis was found in the SA group with decrease in the ratio of the CD4/CD8 subsets in both lung and blood. Neutrophilic alveolitis was also found in 5 patients with SA of long duration. As with other inflammatory diseases, it seems that a subclinical alveolitis is present in SA patients.

[Bronchial cancer with normal thoracic image discovered by endoscopy. Apropos of 11 cases]. / Cancer bronchique à image thoracique normale de découverte endoscopique. A propos de onze observations.

The authors report about eleven cases, distributed over four years, of bronchial cancers with a normal thoracic image that were discovered on bronchial endoscopy. In this context, they sum up the limitations of medical imaging, the undisputed merits of bronchial endoscopy, and the major epidemiological and therapeutic features of this clinical form of bronchial cancer.

[Prolonged effect against exercise-induced bronchospasm: salmeterol versus sodium cromoglycate]. / Protection prolongée du bronchospasme à l'effort: salmétérol versus cromoglycate de sodium.

Nineteen subjects with isolated exercise-induced asthma (FEV1, FEF25-75%, PEFR, FVC greater than 95% predicted values, fall in FEV1 of at least 15% after exercise, typical recent symptoms of exercise-induced asthma, no other treatment) were entered in a multicenter trial carried out in a double blind, double placebo, cross-over manner. After a one-month baseline period, subjects underwent an exercise after inhaling 100 micrograms of salmeterol (n = 12) or 40 mg of sodium cromoglycate (n = 7). Treatments were alternated before the second exercise which took place at least 2 days after the first. Efficacy was assessed by examining changes in FEV1, FEF25-75% after exercise carried out 30 minutes and 7 hours after administering the treatment by comparison with baseline values (assessments done 1, 10 and 30 minutes after exercise, lowest of three values kept for the analysis of each parameter). FEV1 and FEF25-75% were significantly higher 30 minutes after taking salmeterol. Salmeterol was found to be superior to sodium cromoglycate for all parameters 7 hours after administering the drug. Both treatments were well tolerated. This study confirms that the longer duration of effect of salmeterol and its superiority by comparison with the standard treatments of exercise-induced asthma.

[Intrathoracic sarcoidosis associated with tuberculous spondylodiscitis. 2 cases]. / Association sarcoïdose endothoracique et spondylodiscite tuberculeuse. Deux observations.

We report 2 cases of intrathoracic sarcoidosis associated with tuberculous spondylitis. Although such cases are exceptional, one cannot help wondering whether or not sarcoidosis and tuberculosis are interrelated.

[The eosinophilic lung]. / Le poumon éosinophile.

Infiltration of the pulmonary parenchyma by eosinophils is revealed in bronchoalveolar washings. Associated with pulmonary biopsy, this technique has renewed the approach to a classical syndrome. Pulmonary infiltration eosinophilia has multiple etiologies: some are well characterized (parasitic or medicinal), some others pose some nosological or etiopathogenic problems not yet clarified (chronic pulmonary infiltration eosinophilia, vasculitis of the lung). In such difficult cases, use of surgical pulmonary biopsies has to be envisaged.

[Multifocal tuberculosis of bone. Apropos of an exceptional case]. / La tuberculose osseuse multifocale. A propos d'une observation exceptionnelle.

Multifocal tuberculosis of bones (MTB) is exceptional in Europe. To the few cases found in the literature the authors add another case well documented by computerized tomography and nuclear magnetic resonance and remarkable for the number of bone lesions and their coexistence with extra-skeletal lesions. The patient was a 28-year old man native of the Ivory Coast in whom the imaging techniques demonstrated no less than 19 different bone lesions plus an abscess of the iliopsoas muscle and a prevertebral pus collection. The diagnosis of MTB was confirmed by the finding of alcohol- and acid-fast bacilli at needle aspiration of the bone lesions and by the presence of folliculo-caseous Ziehl-stained granuloma on bronchial biopsies. Fourteen months after treatment with specific 4-drug therapy, the outcome is favourable. This case is exceptional by the diffusion of bone lesions and by their association with bronchial lesions due to lymph node fistulization. Modern imaging techniques (CT, NMR), clearly demonstrated the bone lesions and their extent.

[Simultaneous or successive primary multifocal bronchial cancer. Apropos of 12 cases]. / Les cancers bronchiques primitifs multifocaux, simultanés ou successifs. A propos de 12 observations.

Twelve cases of bifocal and bilateral bronchial carcinoma are presented. The two tumours developed successively in 6 cases and simultaneously in 6 cases. Patients' mean age (60 years) was the same as in other lung cancers. Four out of 12 patients produced a history of old ENT or skin cancer. In 11 out of 12 cases the second tumour, undetectable by radiology, was discovered at bronchoscopy. In 2 patients the two tumours had a different histology: adenocarcinoma and squamous cell tumour. In all other cases, both tumours were of the squamous cell type. Treatment of the second contralateral tumour was difficult to determine, especially when the first tumour had been widely excised. However, radiotherapy focused on small endobronchial tumours seemed to give interesting results.

[Data of broncho-alveolar lavage and pulmonary histiocytosis X]. / Données du lavage broncho-alvéolaire et histiocytose X pulmonaire.

The data obtained from broncho-alveolar lavage in 12 cases of pulmonary histiocytosis X explored between 1978 and 1986 are reported. The usefulness of this simple and reliable examination to the diagnosis and follow-up is emphasized. The diagnosis, suggested by radiography of the chest, may be suspected on cytological findings of hypercellularity, macrophage alveolitis and eosinophilia. The occurrence of neutrophilic hypergranulocytosis indicates an unfavourable course towards fibrosis. An increase of cells marked with monoclonal OKT6 antibodies (70% of the cases in this series) contributes to the diagnosis of histiocytosis X and is a good indicator of granuloma activity during the follow-up period. Electron microscopy of the alveolar fluid often confirms the diagnosis by showing the specific organelle: the X granule (60% of the cases in this series), thus avoiding exploratory thoracotomy in many patients.

[Cerebral and pulmonary histiocytosis X. Neurologic manifestations disclosing a pseudotumoral formation on the floor of the 4th ventricle]. / Histiocytose X cérébrale et pulmonaire. Manifestations neurologiques révélatrices d'une formation pseudo-tumorale du plancher du IVe ventricule.

The authors report a case of histiocytosis X which presented with neurological manifestations in a 20 year old man: episodes of headache with vomiting, followed, three months later, by the development of paralysis of the right 6th and 7th cranial nerves associated with nystagmus. The CT scan revealed a tumour-like lesion on the floor of the IVth ventricle with a long axis of 18 mm. The chest x-ray revealed diffuse nodular opacities in the pleural and apical regions with features suggestive of histiocytosis X. The diagnosis was confirmed by surgical biopsy of the typical pulmonary nodules which were rich in histiocytes with X bodies on electron microscopy. The neurological signs disappeared after one month of treatment with Prednisone (1 mg/kg/day) and Vincaleukoblastine (10 mg/week). By the 3rd month, the pulmonary lesions were reduced and the intra-ventricular formation had regressed by 40%. In the authors' series of 29 cases of confirmed histiocytosis X in adults, the present case is the only one with a clinical neurological presentation, apart from 3 cases of diabetes insipidus. A review of the literature confirms the rarity of this type of presentation. The suggestion of the diagnosis by the chest x-ray appearance enabled a dangerous neuro-surgical operation to be avoided.

[Primary pulmonary lymphoma]. / Lymphome pulmonaire primitif.

The authors report 2 cases of primary pulmonary lymphoma. The first case was a 65 year old woman with a diagnosis of lymphocytic lymphoma confirmed by surgical biopsy, after being suspected on the basis of bronchoscopic biopsies and bronchiolo-alveolar aspiration. Twenty one months after the beginning of treatment with chemotherapy and six months after this treatment was completed, the patient was in complete remission. The second patient was a 52 year old man who underwent left inferior lobectomy for a lympho-plasmacytic lymphoma, which was only diagnosed definitively on the operative specimen. The authors discuss the clinical and radiological features and the histological and immunological criteria of primary pulmonary lymphomas (PPL). There are a number of related diseases with varying degrees of malignancy: interstitial lymphocytic pneumonitis (ILP), pseudo-lymphoma, which corresponds to a localised form of ILP, lymphomatoid granulomatosis (LYG) and pulmonary lymphoma, which usually has a lymphocytic or lympho-plasmocytic cell type.

[Mediastinal pseudotumor due to atrial hernia caused by a left pericardial defect]. / Pseudo-tumeur médiastinale par hernie auriculaire au travers d'un défect péricardique gauche.

Radiological examination in a young adult revealed the presence of an opacity facing the second left arch of the heart shadow. This finding associated with data from computed tomography suggested a diagnosis of a thymic tumor. Surgical exploration demonstrated a defect in the left pericardium through which there was a rhythmic protrusion of the auricle and fatty tissue. Aplasia of the pericardium is rarely observed, and usually involves its left side. It results from premature atrophy of the left Cuvier's canal, and is associated with cardiac or pulmonary anomalies in half of the cases. Diagnosis should be suggested by the abnormal appearance of the second left arch, very often clinically asymptomatic, and is confirmed when the creation of a pneumothorax produces a simultaneous pneumopericardium. A thoracic scan can visualize the left auricular hernia beyond the mediastinal limits. However, pericardial aplasia must remain a differential diagnosis of pathological opacities in the middle mediastinum.