A 62 years old, post-menopausal female was admitted to the Internal Medicine Ward due to dyspnoea, cough and sputum of at least 2 months. Shortness of breath, cough and hypoxaemia persisted and the patient was submitt...A 62 years old, post-menopausal female was admitted to the Internal Medicine Ward due to dyspnoea, cough and sputum of at least 2 months. Shortness of breath, cough and hypoxaemia persisted and the patient was submitted to a pulmonary angiogram ct which revealed numerous thin-walled air cysts affecting upper and medial zones of both lungs, typical images of pulmonary lymphangioleiomyomatosis. After discharge to Internal Medicine Consultation Service with Metilprednisolone, the patient was no longer hypoxaemic and remained asymptomatic, even after withdrawal of oral corticosteroid to inhalatory formulation. Further surveillance in short time was scheduled in order to implement rapid imunossupressive treatment when necessary.展开更多
Pulmonary lymphangioleiomyomatosis （PLAM） is a rare disease primarily occurring in women at reproductive age, and characterized by abnormal proliferation of immature smooth muscle cells named lymphangioleiomyomatos...Pulmonary lymphangioleiomyomatosis （PLAM） is a rare disease primarily occurring in women at reproductive age, and characterized by abnormal proliferation of immature smooth muscle cells named lymphangioleiomyomatosis （LAM） cells in the pulmonary lymphatics, blood vessels and airways, resulting in respiratory failure and death.展开更多
文摘A 62 years old, post-menopausal female was admitted to the Internal Medicine Ward due to dyspnoea, cough and sputum of at least 2 months. Shortness of breath, cough and hypoxaemia persisted and the patient was submitted to a pulmonary angiogram ct which revealed numerous thin-walled air cysts affecting upper and medial zones of both lungs, typical images of pulmonary lymphangioleiomyomatosis. After discharge to Internal Medicine Consultation Service with Metilprednisolone, the patient was no longer hypoxaemic and remained asymptomatic, even after withdrawal of oral corticosteroid to inhalatory formulation. Further surveillance in short time was scheduled in order to implement rapid imunossupressive treatment when necessary.
文摘Pulmonary lymphangioleiomyomatosis （PLAM） is a rare disease primarily occurring in women at reproductive age, and characterized by abnormal proliferation of immature smooth muscle cells named lymphangioleiomyomatosis （LAM） cells in the pulmonary lymphatics, blood vessels and airways, resulting in respiratory failure and death.
