Lymphangioleiomyomatosis (LAM) is a rare and progressive pulmonary disease of unknown etiology that almost exclusively affects women. It is characterised by cystic radiological lung pattern and by the possible presence of angiomyolipomas in other sites or organs. Functionally LAM is associated with airway obstruction or restriction and progressive hypoxemia up to chronic respiratory failure. There are no studies, so far, which have investigated whether during sleep these patients show changes in the sleep profile and gas exchange and if these changes are related to disease severity. Aim of the study, prospective and pilot, is to evaluate whether the physiological modification of respiratory mechanics during sleep is associated with polysomnographic alterations in LAM.
Age range
18 Years
Sex
ALL
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prevalence of sleep disorders in LAM
Timeframe: 1 year
correlation between sleep-related disorders and LAM severity measured by spirometry
Timeframe: 1 year
correlation between sleep-related disorders and LAM severity measured by annual decline of forced-expiratory volume at the first second (FEV1)
Timeframe: 1 year
correlation between sleep-related disorders and LAM severity measured by arterial blood gas exchange
Timeframe: 1 year
correlation between sleep-related disorders and clincal parameters
Timeframe: 1 year