Transcription of N-アセチルシステインとピルフェニドンの併用が有効であっ …
1 3 5 2014 713.. N- . 1 .. 73 2008 12 IPF . 5 N- NAC FVC CT . GGO 2010 9 FVC CT GGO . FVC CT GGO NAC .. N- . Idiopathic pulmonary fibrosis, N-Acetylcysteine, Pirfenidone, Combined therapy azathioprine .. 7 3 . idiopathic pulmonary brosis IPF 8 IPF NAC.. primary endpoint . 3 5 1 3 2000 9 IPF. American Thoracic Society ATS /European Respi- NAC . ratory Society ERS NAC 17 . IPF 2 .. IPF 1 . 2011 ATS/ERS/Japa- . nese Respiratory Society JRS /Latin American Tho- . racic Association ALAT IPF . 73 . 3 . The Modified British Medical Re- pirfenidone search Council mMRC 2 . 4 6 . 2008 IPF 20 / 43 .. N- . N-acetylcysteine NAC . prednisolone PSL . 2004 . CT 2008.. 12 mMRC 3 . 143-8541 6-11-1. CT 2009 2 . E-mail: . Received 13 Mar 2014/Accepted 2 Jul 2014 CT HRCT . 714 3 5 2014. 1 . Hematology AST 18 IU/L Blood gas analysis room air . RBC 429 104/ l ALT 10 IU/L pH Hb g/dl LDH 242 U/L PaCO2 Torr Ht ALP 243 IU/L PaO2 Torr Plt 104/ l GT 13 U/L BE mmol/L.
2 WBC 5,900/ l CRP mg/dl HCO3 26 mmol/L. Stab ESR 13 mm/h Seg CK 24 IU/L Spirometry Eos KL-6 976 U/ml FVC 2,920 ml Lym SP-D 335 ng/ml FVC . Baso SP-A 114 ng/ml FEV1 1,760 ml FEV1/FVC . Chemistry Urine FEV1 . TP g/dl Sugar DLCO . T-Bil mg/dl Protein DLCO/VA . Alb g/dl OB . UN 12 mg/dl RBC 1. Cr mg/dl WBC 1. Na 140 mmol/L. K mmol/L. Cl 104 mmol/L. Ca mEq/L. 1 PaO2 . NAC 6 . DLCO CT . 2010 9 . IPF 2009 5 CT 2010. NAC IPF 715. 2 X .. 9 7 6 . 2011 3 . 165 cm kg . 80 /min 18 /min 120/80 mmHg . fine crackles . 6 mMRC 3 . 3 CT . 6 mMRC 2 .. LDH 242 IU/L CRP mg/. 1 . dl PaO2 80 Torr . 6 6 . SpO2 97 SpO2 . 91 330 m KL-6 SP-D NAC. forced vital capacity FVC 2,920 ml . 470 ml FVC DLCO 7 . 6 . 6 X 2 .. C 3 .. KL-6 976 U/ml SP-D 335 ng/ml SP-A 114 ng/ . ml 6 HRCT . ground glass opacity GGO . 1 . FVC 2009 2 5 3 4 2009 2 5 3 . L L L FVC GGO 2009 5 . /3 Mo 2009 5 NAC NAC GGO GGO . L L L FVC /6 2010 9.
3 Mo GGO . L L L FVC 21 /10 Mo 2011 8 11 . 10 2010 . 9 6 idiopathic L L L FVC 19 /6 Mo thrombocytopenic purpura ITP . 716 3 5 2014. 4 HRCT 2009 2 5 ground glass opacity GGO . A 2009 5 NAC GGO A B . GGO C . 2010 9 GGO D . NAC . ITP IPF FVC 95 . 2 600 DLCO 55 NAC. mg PSL 10 mg 9 . FVC L . 1,200 mg 3 IPF . FVC 2013 9 L . PSL NAC . 10 mg/ .. 36 mMRC 2 IPF . FVC DLCO CT - EMT NAC . 1 11 . 2004 9 .. IPF . IPF IPF . 2008 FVC. NAC . TGF- mRNA 17 36 . 53 . TNF- TGF- . TNF- 2 . 10 . NAC 17 . NAC IPF 717. NAC .. NAC . 1 .. 2 2011. CT 25 2 American Thoracic Society. Idiopathic pulmonary FVC brosis: diagnosis and treatment. International con- DLCO sensus statement. American Thoracic Society usual interstitial pneumonia UIP ATS , and the European Respiratory Society combined pulmo- ERS .. Am J Respir Crit Care Med 2000; 161: 646- 64. nary brosis and emphysema CPFE . 3 Raghu G, et al. An official ATS/ERS/JRS/ALAT.
4 CPFE . statement: idiopathic pulmonary brosis: Evidence- . based guidelines for diagnosis and management. FVC . Am J Respir Crit Care Med 2011; 183: 788-824. FVC 4 Azuma A, et al. Double-blind, placebo-controlled tri- CPFE al of pirfenidone in patients with idiopathic pulmo- nary brosis Am J Respir Crit Care Med 2005; 171: 1040-7. ITP 5 Taniguchi H, et al. Pirfenidone Clinical Study Group 1 in Japan: Pirfenidone in idiopathic pulmonary bro- COPD sis. Eur Respir J 2010; 35: 821-9. 6 Noble PW, et al. Pirfenidone in patients with idio- pathic pulmonary brosis CAPACITY : two ran- domized trials. Lancet 2011; 377: 1760-9. NAC . 7 Demedts M, et al; IFIGENIA Study Group. High- . dose acetylcysteine in idiopathic pulmonary bro- NAC . sis. N Engl J Med 2005; 353: 2229-42.. 8 Idiopathic Pulmonary Fibrosis Clinical Research Network, Raghu G, et al. Prednisone, azathioprine, 2 and N-acetylcysteine for pulmonary brosis.
5 N Engl J Med 2012; 366: 1968-77. NAC 9 Homma S, et al. E cacy of inhaled N-acetylcysteine monotherapy in patients with early stage idiopathic IPF NAC pulmonary brosis. Respirology 2012; 17: 467-77. 10 1 . 2009; 45: 1882-7.. 11 Felton VM, et al. N-acetylcysteine inhibits alveolar . epithelial-mesenchymal transition. Am J Physiol Lung Cell Mol Physiol 2009; 297: 805-12. COI con icts of interest . 718 3 5 2014. Abstract Effectiveness of combined therapy with pirfenidone and inhaled N-acetylcysteine for idiopathic pulmonary fibrosis: A case report Susumu Sakamoto, Kyoko Gocho, Keita Satoh, Keishi Sugino, Kazutoshi Isobe and Sakae Homma Department of Respiratory Medicine, Toho University Omori Medical Center A 73-year-old man was referred to our hospital in February 2009 for cough and dyspnea. A diagnosis of idio- pathic pulmonary fibrosis was made on the basis of findings from chest computed tomography CT and a pul- monary function test PFT.
6 The patient was started on inhaled N-acetylcysteine in May 2009, and chest CT and PFT findings L; change in forced vital capacity FVC , had improved at 6 months. However, PFT and CT findings had worsened L; FVC, 21 at 17 months after starting inhaled N-acetylcyste- ine therapy, and pirfenidone treatment was added to inhaled NAC therapy in September 2010. CT and PFT find- ings L; FVC, 19 were markedly improved at 6 months after beginning combination therapy and remained at 36 months. This is the first report of successful treatment for disease progression of idiopathic pul- monary fibrosis with combination therapy comprising N-acetylcysteine and pirfenidone.
