[Pulmonary lymphangioleiomyomatosis: From pathogenesis to management]. / Lymphangioléiomyomatose pulmonaire : de la physiopathologie à la prise en charge.

INTRODUCTION: Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease affecting mainly young women. BACKGROUND: The respiratory manifestations are characterized by a progressive cystic destruction of the lung parenchyma. Extrapulmonary involvement includes benign renal tumours called angiomyolipomas and abdominal lymphatic masses called lymphangioleiomyomas. At the pathological level, the cellular proliferation found in LAM is in part due to the presence of mutations in the tumour suppressor genes TSC1 and TSC2 (Tuberous Sclerosis Complex). These mutations lead to the activation of the mTOR pathway, which is currently the main therapeutic target. mTOR inhibitors such as sirolimus or everolimus have shown a beneficial effect on the decline in pulmonary function and a reduction of angiomyolipoma size, but are necessary in only some patients. PERSPECTIVES: LAM cells have migratory properties mediated by the formation of new lymphatic vessels. They are also able to secrete metalloproteases, which enhance their invasiveness. Moreover, the expression of estrogen and progesterone receptors by LAM cells suggests a possible role for sex hormones in the pathogenesis of the disease. CONCLUSION: A better understanding of mTOR-independent mechanisms would allow the development of novel therapeutic approaches.

[Staphylococcus aureus broncho-pulmonary infections]. / Infections broncho-pulmonaires à Staphylococcus aureus.

Staphylococcus aureus accounts for 2-5% of the etiologies of community-acquired pneumonia. These infections occur mainly in elderly patients with comorbidity, after a respiratory viral infection. S. aureus could also be responsible for necrotizing pneumonia, which occurs in young subjects, also after flu. Necrotizing pneumonia are associated with the production of a particular staphylococcal toxin called Panton-Valentine leukocidin, responsible for pulmonary focal necrosis, occurrence haemoptysis, leucopenia, and death. In Europe, these strains are still predominantly sensitive to anti-staphylococcal penicillin, which must be used at high dosage intravenously in combination with an antibiotic that reduces toxin production such as clindamycin, and intravenous immunoglobulin in severe cases. The mortality rate is estimated at 50%. In addition, S. aureus is one of the pathogens involved in early respiratory infections in cystic fibrosis patients, in whom methicillin resistance plays an important prognostic role. However, the involvement of S. aureus in COPD exacerbations is rare. Finally, S. aureus represents 20 to 30% of cases of hospital-acquired pneumonia, including ventilator-associated pneumonia. In these cases, methicillin-resistance is common and requires the use of glycopeptides or linezolid. The place of new anti-staphylococcal antibiotics such as new generation cephalosporins or tigecyclin remains to be defined.