Effectiveness of pulmonary rehabilitation in lymphangioleiomyomatosis

Abstract

Lymphangioleiomyomatosis (LAM) is a cystic lung disease frequently associated with reduced exercise capacity, mostly affecting young women. The disease is characterized by progressive pulmonary cystic change, recurrent pneumothorax, chylous pleural collections, and in most cases, progressive respiratory failure. Diagnosis is made by a combination of clinical features and computed tomography scanning or, with lung biopsy. We present a case report of a young woman who suspected of LAM and diagnosed with cystic lung disease, admitted to the intensive care unit in view of worsening hypoxia associated with pneumothorax. She was on ventilator support and placement of repeated intercostal drains and had a tracheostomy to step down to bilevel positive airway pressure support, after the medical stabilization. She was referred for pulmonary rehabilitation, in which she showed good outcomes and recovered well.

Keywords

• hormonal therapy
• lymphangioleiomyomatosis
• pulmonary rehabilitation