特发性肺纤维化治疗新进展
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摘要
<正>特发性肺纤维化(idiopathic pulmonary fibrosis,IPF)是一种病因未明的慢性间质性肺疾病(interstitial lung diseases,ILD),进行性纤维化过程引起肺结构和功能的广泛改变。IPF是ILD中最常见的类型,按照目前的诊断标准,估计年发病率为(0.22～8.8)/10万人。IPF 5年生存率为20%～25%。因此,IPF被称为不是癌症的癌症~([1])。
引文
1 Skold CM, Bendstrup E, Myllarniemi M, et al. Treatment of idiopathic pulmonary fibrosis:a position paper from a Nordic expert group. J Intern Med, 2016, 281(2):149-166.
2 Peljto AL, Zhang Y, Fingerlin TE, et al. Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis. JAMA, 2013, 309(21):2232-2239.
3 Andersson-Sjoland A, de Alba CG, Nihlberg K, et al. Fibrocytes are a potential source of lung fibroblasts in idiopathic pulmonary fibrosis. Int J Biochem Cell Biol, 2008, 40(10):2129-2140.
4 Maher TM, Wells AU, Laurent GJ. Idiopathic pulmonary fibrosis:multiple causes and multiple mechanisms?. Eur Respir J, 2007,30(5):835-839.
5 Pandit KV, Milosevic J, Kaminski N. MicroRNAs in idiopathic pulmonary fibrosis. Transl Res, 2011.157(4):191-199.
6 Thabut G, Mal H, Castier Y, et al. Survival benefit of lung transplantation for patients with idiopathic pulmonary fibrosis.J Thorac Cardiovasc Surg, 2003, 126(2):469-475.
7 Schaffer JM, Singh SK, Reitz BA, et al. Single-vs double-lung transplantation in patients with chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis since the implementation of lung allocation based on medical need. JAMA,2015, 313(9):936-948.
8 Nishiyama O, Miyajima H, Fukai Y, et al. Effect of ambulatory oxygen on exertional dyspnea in IPF patients without resting hypoxemia. Respir Med, 2013,107(8):1241-1246.
9 Douglas WW, Ryu JH, Schroeder DR. Idiopathic pulmonary fibrosis:impact of oxygen and colchicine, prednisone, or no therapy on survival. Am J Respir Crit Care Med, 2000, 161(4 Pt 1):1172-1178.
10 Bendstrup E, Hyldgaard C, Altraja A, et al. Organisation of diagnosis and treatment of idiopathic pulmonary fibrosis and other interstitial lung diseases in the Nordic countries. Eur Clin Respir J,2015,2(1):28348.
11 Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev, 2014, 10(10):1-55.
12 Vainshelboim B, Oliveira J, Fox BD, et al. Long-Term effects of a12-week exercise training program on clinical outcomes in idiopathic pulmonary fibrosis. Lung, 2015, 193(3):345-354.
13 Ryerson CJ, Abbritti M, Ley B, et al. Cough predicts prognosis in idiopathic pulmonary fibrosis. Respirology, 2011, 16(6):969-975.
14 Kohberg C, Andersen CU, Bendstrup E. Opioids:an unexplored option for treatment of dyspnea in IPF. Eur Clin Respir J, 2016, 3:30629.
15 Sun T, Liu J, Zhao DW. Efficacy of N-acetylcysteine in idiopathic pulmonary fibrosis:a systematic review and meta-analysis.Medicine(Baltimore), 2016, 95(19):3629-3636.
16 Demedts M, Behr J, Buhl R, et al. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med, 2006, 65(5):2229-2242.
17 The Idiopathic Pulmonary Fibrosis Clinical Research Network;Raghu G, Anstrom KJ, King TE Jr, et al. Prednisone, azathioprine,and N-acetylcysteine for pulmonary fibrosis. N Engl J Med, 2012,366(21):1968-1977.
18 The Idiopathic Pulmonary Fibrosis Clinical Research Network;Martinez FJ, de Andrade JA, Anstrom KJ, et al. Randomized trial of N-acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med,2014, 370(22):2093-2101.
19 Oltmanns U, Kahn N, Palmowski K, et al. Pirfenidone in idiopathic pulmonary fibrosis:real-life experience from a German tertiary referral center for interstitial lung diseases. Respiration, 2014,88(3):199-207.
20 Sakamoto S, Muramatsu Y, Satoh K, et al. Effectiveness of combined therapy with pirfenidone and inhaled N-acetylcysteine for advanced idiopathic pulmonary fibrosis:a case-control study.Respirology, 2015, 20(3):445-452.
21 Homma S, Azuma A, Taniguchi H, et al. Efficacy of inhaled N-acetylcysteine monotherapy in patients with early stage idiopathic pulmonary fibrosis. Respirology, 2012,17(3):467-477.
22 Oldham JM, Ma SF, Martinez FJ, et al. TOLLIP, MUC5B, and the response to N-acetylcysteine among individuals with idiopathic pulmonary fibrosis,. Am J Respir Crit Care Med, 2015, 192(12):1475-1482.
23 Bando M. Pirfenidone:clinical trials and clinical practice in patients with idiopathic pulmonary fibrosis. Respir Investig, 2016,54(5):298-304.
24 Azuma A, Nukiwa T, Tsuboi E, et al. Double-blind, placebocontrolled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med, 2005, 171(9):1040-1047.
25 Taniguchi H, Ebina M, Kondoh Y, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J, 2010, 35(4):821-829.
26 Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis(CAPACITY):two randomised trials. Lancet, 2011, 377(9779):1760-1769.
27 King TE Jr, Bradford WZ, Castro-Bernardini S, et al. A phase 3trial of pirfenidone in patients with idiopathic pulmonary fibrosis.N Engl J Med, 2014, 370(22):2083-2092.
28 Aravena C, Labarca G, Venegas C, et al. Correction:pirfenidone for idiopathic pulmonary fibrosis:a systematic review and metaanalysis. PLoS One, 2015, 10(10):0140288.
29 Costabel U, Albera C, Bradford WZ, et al. Analysis of lung function and survival in RECAP:an open-label extension study of pirfenidone in patients with idiopathic pulmonary fibrosis.Sarcoidosis Vasc Diffuse Lung Dis, 2014, 31(3):198-205.
30 Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med, 2014,370(22):2071-2082.
31 Richeldi L, Costabel U, Selman M, et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med,2011,365(12):1079-1087.
32 Tomioka H, Takada H. Treatment with nintedanib for acute exacerbation of idiopathic pulmonary fibrosis. Respirol Case Rep,2017, 5(2):e00215.
2 Peljto AL, Zhang Y, Fingerlin TE, et al. Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis. JAMA, 2013, 309(21):2232-2239.
3 Andersson-Sjoland A, de Alba CG, Nihlberg K, et al. Fibrocytes are a potential source of lung fibroblasts in idiopathic pulmonary fibrosis. Int J Biochem Cell Biol, 2008, 40(10):2129-2140.
4 Maher TM, Wells AU, Laurent GJ. Idiopathic pulmonary fibrosis:multiple causes and multiple mechanisms?. Eur Respir J, 2007,30(5):835-839.
5 Pandit KV, Milosevic J, Kaminski N. MicroRNAs in idiopathic pulmonary fibrosis. Transl Res, 2011.157(4):191-199.
6 Thabut G, Mal H, Castier Y, et al. Survival benefit of lung transplantation for patients with idiopathic pulmonary fibrosis.J Thorac Cardiovasc Surg, 2003, 126(2):469-475.
7 Schaffer JM, Singh SK, Reitz BA, et al. Single-vs double-lung transplantation in patients with chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis since the implementation of lung allocation based on medical need. JAMA,2015, 313(9):936-948.
8 Nishiyama O, Miyajima H, Fukai Y, et al. Effect of ambulatory oxygen on exertional dyspnea in IPF patients without resting hypoxemia. Respir Med, 2013,107(8):1241-1246.
9 Douglas WW, Ryu JH, Schroeder DR. Idiopathic pulmonary fibrosis:impact of oxygen and colchicine, prednisone, or no therapy on survival. Am J Respir Crit Care Med, 2000, 161(4 Pt 1):1172-1178.
10 Bendstrup E, Hyldgaard C, Altraja A, et al. Organisation of diagnosis and treatment of idiopathic pulmonary fibrosis and other interstitial lung diseases in the Nordic countries. Eur Clin Respir J,2015,2(1):28348.
11 Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev, 2014, 10(10):1-55.
12 Vainshelboim B, Oliveira J, Fox BD, et al. Long-Term effects of a12-week exercise training program on clinical outcomes in idiopathic pulmonary fibrosis. Lung, 2015, 193(3):345-354.
13 Ryerson CJ, Abbritti M, Ley B, et al. Cough predicts prognosis in idiopathic pulmonary fibrosis. Respirology, 2011, 16(6):969-975.
14 Kohberg C, Andersen CU, Bendstrup E. Opioids:an unexplored option for treatment of dyspnea in IPF. Eur Clin Respir J, 2016, 3:30629.
15 Sun T, Liu J, Zhao DW. Efficacy of N-acetylcysteine in idiopathic pulmonary fibrosis:a systematic review and meta-analysis.Medicine(Baltimore), 2016, 95(19):3629-3636.
16 Demedts M, Behr J, Buhl R, et al. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med, 2006, 65(5):2229-2242.
17 The Idiopathic Pulmonary Fibrosis Clinical Research Network;Raghu G, Anstrom KJ, King TE Jr, et al. Prednisone, azathioprine,and N-acetylcysteine for pulmonary fibrosis. N Engl J Med, 2012,366(21):1968-1977.
18 The Idiopathic Pulmonary Fibrosis Clinical Research Network;Martinez FJ, de Andrade JA, Anstrom KJ, et al. Randomized trial of N-acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med,2014, 370(22):2093-2101.
19 Oltmanns U, Kahn N, Palmowski K, et al. Pirfenidone in idiopathic pulmonary fibrosis:real-life experience from a German tertiary referral center for interstitial lung diseases. Respiration, 2014,88(3):199-207.
20 Sakamoto S, Muramatsu Y, Satoh K, et al. Effectiveness of combined therapy with pirfenidone and inhaled N-acetylcysteine for advanced idiopathic pulmonary fibrosis:a case-control study.Respirology, 2015, 20(3):445-452.
21 Homma S, Azuma A, Taniguchi H, et al. Efficacy of inhaled N-acetylcysteine monotherapy in patients with early stage idiopathic pulmonary fibrosis. Respirology, 2012,17(3):467-477.
22 Oldham JM, Ma SF, Martinez FJ, et al. TOLLIP, MUC5B, and the response to N-acetylcysteine among individuals with idiopathic pulmonary fibrosis,. Am J Respir Crit Care Med, 2015, 192(12):1475-1482.
23 Bando M. Pirfenidone:clinical trials and clinical practice in patients with idiopathic pulmonary fibrosis. Respir Investig, 2016,54(5):298-304.
24 Azuma A, Nukiwa T, Tsuboi E, et al. Double-blind, placebocontrolled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med, 2005, 171(9):1040-1047.
25 Taniguchi H, Ebina M, Kondoh Y, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J, 2010, 35(4):821-829.
26 Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis(CAPACITY):two randomised trials. Lancet, 2011, 377(9779):1760-1769.
27 King TE Jr, Bradford WZ, Castro-Bernardini S, et al. A phase 3trial of pirfenidone in patients with idiopathic pulmonary fibrosis.N Engl J Med, 2014, 370(22):2083-2092.
28 Aravena C, Labarca G, Venegas C, et al. Correction:pirfenidone for idiopathic pulmonary fibrosis:a systematic review and metaanalysis. PLoS One, 2015, 10(10):0140288.
29 Costabel U, Albera C, Bradford WZ, et al. Analysis of lung function and survival in RECAP:an open-label extension study of pirfenidone in patients with idiopathic pulmonary fibrosis.Sarcoidosis Vasc Diffuse Lung Dis, 2014, 31(3):198-205.
30 Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med, 2014,370(22):2071-2082.
31 Richeldi L, Costabel U, Selman M, et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med,2011,365(12):1079-1087.
32 Tomioka H, Takada H. Treatment with nintedanib for acute exacerbation of idiopathic pulmonary fibrosis. Respirol Case Rep,2017, 5(2):e00215.