Disclosed is the use of N-(5-hydroxy-2,4-ditert-butyl-phenyl)-4-oxo-1H-quinoline-3-carboxamide, or a pharmaceutically acceptable salt thereof, in the manufacture of a medicament for treating or lessening the severity of a disease in a patient, wherein said disease is selected from hereditary emphysema, hereditary hemochromatosis, coagulation-fibrinolysis deficiencies, lipid processing deficiencies, lysosomal storage diseases, polyglutamine neurological disorders, and spongiform encephalopathies, and specifically wherein the disease is COPD, dry-eye disease, or emphysema. Also disclosed is the use of N-(5-hydroxy-2,4-ditert-butyl-phenyl)-4-oxo-1H-quinoline-3-carboxamide, or a pharmaceutically acceptable salt thereof, in the manufacture of a medicament for treating or lessening the severity of cystic fibrosis, wherein the patient possesses a class III, class IV, or class V CFTR mutation, wherein said mutation is not a DF508 CFTR mutation or a G551D CFTR mutation.
