The proposed mechanisms for the development of airway obstruction in Fabry disease include loss of lung elastic recoil, hyperreactivity (bronchospasm), airway inflammation and airway narrowing related to accumulation of glycosphingolipid and hyperplasia in airway epithelial and smooth muscle cells [[11], [12], [13]]. However, a putative mechanism involving airway hyperreactivity and bronchospasm was not supported in one study. None of the patients subjected to methacholine challenge testing exhibited a positive response [11].