High-Dose Acetylcysteine in Idiopathic Pulmonary Fibrosis

• Published in: The New England journal of medicine Vol. 353; no. 21; pp. 2229 - 2242
• Main Authors: Demedts, Maurits, Behr, Juergen, Buhl, Roland, Costabel, Ulrich, Dekhuijzen, Richard, Jansen, Henk M, MacNee, William, Thomeer, Michiel, Wallaert, Benoit, Laurent, François, Nicholson, Andrew G, Verbeken, Eric K, Verschakelen, Johny, Flower, Christopher D.R, Capron, Frédérique, Petruzzelli, Stefano, De Vuyst, Paul, van den Bosch, Jules M.M, Rodriguez-Becerra, Eulogio, Corvasce, Giuseppina, Lankhorst, Ida, Sardina, Marco, Montanari, Mauro
• Format: Journal Article
• Language: English
• Published: Boston, MA Massachusetts Medical Society 24.11.2005
• Subjects: Acetylcysteine - administration & dosage; Acetylcysteine - adverse effects; Acetylcysteine - pharmacology; Aged; Anti-Inflammatory Agents - therapeutic use; Antioxidants - administration & dosage; Antioxidants - adverse effects; Antioxidants - pharmacology; Azathioprine - therapeutic use; Biological and medical sciences; Double-Blind Method; Drug dosages; Drug therapy; Drug Therapy, Combination; Exercise Tolerance - drug effects; Female; General aspects; Humans; Immunosuppressive Agents - therapeutic use; Lungs; Male; Medical sciences; Middle Aged; Pneumology; Pneumonia; Prednisone - therapeutic use; Pulmonary Diffusing Capacity - drug effects; Pulmonary Fibrosis - drug therapy; Pulmonary Fibrosis - mortality; Pulmonary Fibrosis - physiopathology; Respiratory system : syndromes and miscellaneous diseases; Vital Capacity - drug effects; Medicine; Lung disease; Mucolytic; Thiol; Pulmonary fibrosis; Respiratory disease; Idiopathic; Acetylcysteine; Antioxidant; High dose
• ISSN: 0028-4793; 1533-4406
• DOI: 10.1056/NEJMoa042976

Idiopathic pulmonary fibrosis is a progressive disorder characterized by loss of lung function; there is no effective treatment. In this multinational, randomized, placebo-controlled trial with all patients receiving azathioprine and prednisone, acetylcysteine was shown to slow the rate of deterioration of lung function. There was no effect on survival. In patients receiving azathioprine and prednisone, acetylcysteine was shown to slow the rate of deterioration of lung function. There was no effect on survival. Idiopathic pulmonary fibrosis is a chronic progressive interstitial pneumonia with a poor prognosis. 1 – 4 It has been proposed that a pathogenetic mechanism of idiopathic pulmonary fibrosis is repeated lung injury, with aberrant progressive fibrotic reaction. 5 – 9 If this is the case, it may explain why treatment with corticosteroids and immunosuppressive agents results in only slight therapeutic benefit. 3 , 10 – 12 An oxidant–antioxidant imbalance may contribute to the disease process in idiopathic pulmonary fibrosis. 13 – 20 Acetylcysteine, a precursor of the major antioxidant glutathione, given at a daily dose of 1800 mg, has been shown to restore depleted pulmonary glutathione levels 16 – 19 and . . .