[Pulmonary complications of Chronic Granulomatous Disease]. / Complications pulmonaires de la granulomatose septique chronique.

Chronic Granulomatosis Disease (CGD) is an inherited immune deficiency due to a mutation in the genes coding for the subunits of the NADPH oxidase enzyme that affects the oxidative capacity of phagocytic cells. It is characterized by increased susceptibility to bacterial and fungal infections, particularly Aspergillus, as well as complications associated with hyperinflammation and granulomatous tissue infiltration. There exist two types of frequently encountered pulmonary manifestations: (1) due to their being initially pauci-symptomatic, possibly life-threatening infectious complications are often discovered at a late stage. Though their incidence has decreased through systematic anti-bacterial and anti-fungal prophylaxis, they remain a major cause of morbidity and mortality; (2) inflammatory complications consist in persistent granulomatous mass or interstitial pneumoniae, eventually requiring immunosuppressive treatment. Pulmonary complications recurring since infancy generate parenchymal and bronchial sequelae that impact functional prognosis. Hematopoietic stem cell allograft is a curative treatment; it is arguably life-sustaining and may limit the morbidity of the disease. As a result of improved pediatric management, life expectancy has increased dramatically. That said, new challenges have appeared with regard to adults: difficulties of compliance, increased inflammatory manifestations, acquired resistance to anti-infectious therapies. These different developments underscore the importance of the transition period and the need for multidisciplinary management.

[Angiosarcoma mimicking recurrent pulmonary embolism]. / Un angiosarcome mimant une embolie pulmonaire récidivante.

INTRODUCTION: Pulmonary artery sarcoma is a rare disease with non-specific symptoms. The clinical and radiological presentation can mimic pulmonary embolism with chronic thromboembolic pulmonary hypertension. Management is essentially surgical but the prognosis remains poor. CASE REPORT: A patient presented with symptoms of pulmonary embolism. Despite vitamin K antagonist therapy, he suffered from extension of the endovascular defects and his pulmonary hypertension increased. Suspicious results of positron emission tomography suggested the diagnosis of pulmonary artery sarcoma that was confirmed by surgery. However, the outcome was unfavourable, leading to death of the patient. CONCLUSION: This case reinforces the idea that the clinical and tomodensitometric presentations of pulmonary arterial sarcoma and chronic thromboembolic pulmonary hypertension are similar. The positron emission tomography seems to be a key to distinguishing these two diagnoses.