[Pulmonary alveolar proteinosis]. / La protéinose alvéolaire pulmonaire.

Alveolar proteinosis (AP) is a rare disease characterized by alveolar accumulation of surfactant components, which impairs gas exchange. AP is classified into three groups: auto-immune AP defined by the presence of plasma autoantibodies anti-GM-CSF, the most frequent form (90% of all AP); secondary AP, mainly occurring as a consequence of haematological diseases, or following on from toxic inhalation or infections, and genetic AP, which affects almost exclusively children. AP diagnosis is suspected where chest CT-scan demonstrates interstitial lung disease with a crazy paving aspect; and confirmed by bronchoalveolar lavage, which has a milky appearance and contains periodic acid Schiff positive proteinaceous alveolar deposits. The use of surgical lung biopsy to confirm AP is less frequent nowadays. In this context, positive antibodies against GM-CSF indicates an auto-immune etiology of the AP. Concerning management, whole lung lavage is the gold standard therapy. In refractory AP, new treatments are available such as subcutaneous or inhaled GM-CSF supplementation, or rituximab infusions. The clinical course is unpredictable. Spontaneous improvement or even cure can occur, and the 5-year actuarial survival is 95%. The most frequent complications are infectious etiology.

[Pulmonary alveolar proteinosis]. / Lipoprotéinose alvéolaire pulmonaire.

Pulmonary alveolar proteinosis (PAP) is a rare, diffuse infiltrative lung disease of unknown etiology characterized by accumulation of abnormal lung surfactant in the alveoli and distal airways. Although it was first described in 1958, few large scale studies have been published. The diagnosis is conventionally based on lung biopsy and the value of less invasive procedures such as bronchoalveolar lavage (BAL) remains controversial. A multi-center retrospective study was conducted in an attempt to update epidemiological, clinical and long term outcome and to assess the usefulness of chest CT scan and BAL in the management of a large homogeneous series of 41 patients. Inclusion criteria required a clinical picture compatible with PAP and either histological confirmation, or a BAL with a milky macroscopic appearance coupled with positive staining to PAS. Mean age was 48.8 years with a 4: 1 male predominance. The prevalence of smokers (80%) and occupational exposure to various inhaled dusts (39%) was high. The clinical, roentgenographic and functional features were in keeping with previous series. Worsening dyspnea was the most common symptom. Other common manifestations were dry cough, recurrent lung infections, weight loss, finger clubbing, crackles and cyanosis. LDH serum levels were increased in 51% of cases. Lung function tests usually showed a mild restrictive ventilatory defect with hypoxemia and reduced DLCO. Chest X ray revealed bilateral ground glass opacities with consolidation or air bronchogram involving peri-hilar or lower lobes. Chest CT scan, performed in 38 patients, revealed a similar pattern in most of them with diffuse intra-alveolar ground glass opacities (33/38) and interlobular thickening (35/38), resulting in a crazy paving appearance in the typical geographic pattern (32/38). This latter aspect was highly suggestive of the diagnosis. Segmental BAL led to a definitive diagnosis in 62% and lung biopsy was only required in 38% of cases. These data suggest that careful BAL analysis can be sufficient to provide a diagnosis without the need for a lung biopsy. During follow-up, spontaneous remission or improvement was reported in 26%. Segmental (n=3) or whole lung lavage (n=22) was required in 62.5% with a favorable course (complete remission 37%, improvement 34%, stable course 21%) in 92% of the cases. In this series the overall prognosis for PAP remained good, although unpredictable.

[Idiopathic diffuse lung diseases with alveolar infiltration]. / Pneumopathies diffuses idiopathiques avec comblement alvéolaire.

Diffuse idiopathic pneumonia with alveolar filling gather disorders whose clinical and radiological aspects are varied. It is important to precisely identify them because of the prognostic and therapeutic implications suitable for each one of them. Among these, it is necessary to distinguish the eosinophilic pneumonias, wether acute or chronic, which are characterized by circulating and (or) alveolar hypereosinophilies; the diffuse alveolar haemorrhages whose potential severity requires an early recognition in order to institute a treatment without delay; the pulmonary alveolar phospholipoproteinosis can be easily evoked because of their particular radiological features, associated with a lactescent aspect of bronchoalveolar lavage. Lastly, bronchiolo-alveolar carcinoma and more exceptionally the pulmonary alveolar microlithiasis must be considered.

[Respiratory syncytial virus pneumonia in four immunocompromised adults]. / Pneumopathie interstitielle à virus respiratoire syncytial chez quatre adultes immunodéprimés.

INTRODUCTION: In hematologic malignancies, respiratory syncytial viral infections can be explained by neutropenia, and cellular and humoral immunodepression, and may cause severe respiratory infections. EXEGESIS: Four patients with hematologic malignancies developed a severe respiratory syncytial virus infection. Three of them had previously received autologous bone marrow transplantation (ABMT). Progress was favorable for three patients. One patient died of acute respiratory failure. CONCLUSION: When such patients present with respiratory symptoms, especially during the winter months, they should be screened for RSV. Bronchoalveolar lavage allowed quick and accurate diagnosis by immunofluorescence. Treatment with nebulized ribavirin is controversial. Its use may be interesting in patients with high-risk factors (intensive chemotherapy, ABMT, diffuse pneumonia with hypoxemia).

Tracheobronchial stenting in patients with esophageal cancer involving the central airways.

BACKGROUND AND STUDY AIMS: Locoregional progression of esophageal cancer can result in respiratory distress aving to tracheoesophageal (T-E) fistula or central airways stenosis. We report our experience of airway stenting in 51 patients with esophageal carcinoma involving the central airways. PATIENTS AND METHODS: All data were recorded retrospectively. Fifty-one patients (44 men and seven women), with a mean age of 58.6 years, were analyzed. All had an esophageal squamous cell carcinoma. Severe respiratory impairment due to tumor invasion or to a tracheobronchial fistula (n= 14) was present in all patients. Nine of the 14 patients with fistula had dysphagia. Among the 37 patients without fistula, 19 had dysphagia. RESULTS: Sixty-six tracheobronchial stents were inserted in 51 patients: 65 Dumon stents and one Wallstent. Forty stents were implanted in the trachea, 16 in the left main bronchus and 10 in the right main bronchus. In 47 patients there was a significant improvement of respiratory symptoms. Esophageal intubation, carried out in nine patients, allowed eating and drinking in all cases. Mean survival was 107.7 days, with the longest follow-up 587 days. There was no difference between mean survival in the patients with fistulae (103.3+/-days) and the others (109.3+/-days). In two cases stent placement was responsible for death (massive hemoptysis and pneumonia). The main complications were migration (n=6), granuloma (n=2), pneumonia (n=2) and pneumothorax (n = 2). In 13 patients tumor progression in the airways was noted from one to 11 months after stenting, inducing a relapse of dyspnea. CONCLUSIONS: Complications are easily detected by the appearance of respiratory symptoms and do not necessitate systematic flexible bronchoscopy, but only preventive measures such as regular aerosol therapy, adapted respiratory physiotherapy and regular clinical follow-up.

Acute respiratory failure caused by secondary alveolar proteinosis in a patient with acute myeloid leukemia: a case report.

Pulmonary alvelolar proteinosis (PAP) is a rare cause of chronic respiratory failure due to progressive alveolar accumulation of a periodic acid-schiff (PAS) positive proteinaceous material. In some cases, the rapid accumulation of intra-alveolar material leads to acute respiratory failure (ARF). We report the causative role of secondary PAP in the case of a 26-year-old man with acute myeloid leukemia who developed fever, increased serum lactate dehydrogenase level and ARF, and required mechanical ventilation. The diagnosis of PAP was established by the examination of material obtained by bronchoalveolar lavage (BAL). Respiratory improvement occurred several days after the patient had recovered from neutropenia. This report underlines the importance of the early diagnosis of PAP as a potential cause of ARF in leukemic patients. Adequate stain on BAL fluid provides the diagnosis and avoids repeated invasive procedures and inappropriate treatments.

Alveolar haemorrhage revealing epithelioid haemangioendothelioma.

Epithelioid haemangioendothelioma of the pulmonary vessels is a rare neoplasm which usually has a minimal clinical expression. The present case report describes an exceptional case with severe inaugural alveolar haemorrhage which led to death by acute respiratory failure within a few weeks.

Serum and tissue distribution of a fragment of cytokeratin 19 (cyfra 21-1) in lung cancer patients.

A sensitive and relatively specific tumoral marker for lung epidermoid carcinomas could be used to identify patients likely to benefit from new therapeutic protocols. The cyfra 21-1 fragment of cytokeratin 19 has raised much hope in this regard amongst both technologists and clinicians. In a study of 195 subjects, we have shown by means of a serum assay that the usual cut-off value for this marker (3.3 ng/ml) can be lowered to 1.5 ng/ml without loss of specificity, and with an increase in sensitivity. There was a good correlation between serum marker level and tumor extension, but though cyfra 21-1 was not predictive of the suitability of a patient for surgery. A decrease of cyfra-21-1 was observed after complete resection of the tumor. There was no relation between serum assay results and immunohistochemical findings.

[Interstitial pneumonia and mitomycin C. Apropos of a case with analysis of the bronchoalveolar lavage]. / Pneumopathie interstitielle et mitomycine C. A propos d'un cas avec étude du lavage broncho-alvéolaire.

Mitomycin C is an antibiotic used for its alkylizing effect in the treatment of broncogenic cancer. Haematologic, renal or pulmonary complications are sometimes severe. We report a case in a patient with poorly differentiated epidermoid bronchogenic carcinoma who developed clinical and radiological signs of bilateral infiltrating pneumonia due to mitomycine. This observation emphasizes the importance of bronchoalveolar lavage which implicated the immunological toxicity of mitomycine and explained the efficacity of corticosteroid treatment. Pulmonary lesions due to mitomycine were discussed.