An autopsied case of pulmonary lymphangioleiomyomatosis observed for 16 years

A 46-year-old woman complained of dyspnea on exertion. She was diagnosed as having lymphangioleiomyomatosis based on the results of open lung biopsied specimens, and hormonal therapy was begun at the age of 35 at a previous hospital. After that, she was admitted to our hospital because of right pneumothorax and treated by pleurodesis. Uvulectomy was also performed. Home oxygen therapy was started from 41 years old. However, chronic respiratory failure gradually progressed. She was urgently admitted to our hospital because of pneumonia with hypercapnic respiratory failure and mechanical ventilation was started. After pneumonia was improved, management by ventilator was continued. Many chylous ascites had occurred since 50 years old. Malnutrition had gradually progressed. Pneumonia and sepsis were repeated in spite of empiric antibiotic therapy. According to the autopsy results, she died of multiple organ failure due to sepsis at the age of 51.