特发性肺纤维化：修订间差异

Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis
	特发性肺纤维化的電腦斷層影像
类型	特發性間質性肺炎、肺纖維化
分类和外部资源
醫學專科	胸腔醫學
ICD-11	CB03.4
ICD-10	J84.1
ICD-9-CM	516.3
OMIM	178500
DiseasesDB	4815
MedlinePlus	000069
eMedicine	radio/873
MeSH	D011658
Orphanet	2032
	[编辑此条目的维基数据]

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2016年6月14日 (二) 13:47的版本

特发性肺纤维化(Idiopathic Pulmonary Fibrosis, IPF)又称隐源性纤维化肺泡炎，弥漫性纤维化肺泡炎，间质性弥漫性肺纤维化為特發性间质性肺炎中最常見之一種，是无明显原因的，进行性的肺纤维化。肺间质的广泛纤维化形成而肺组织增厚，造成不可逆转地丧失肺组织氧气交换的能力。是一致命的呼吸系统疾病。最常发生在50至70岁的老年人。

起因

病因不清。
风险因素可能包括吸烟、抗忧郁剂、胃食道逆流征、职业粉尘接触，病毒感染^[1] 和先天性(家族性肺纤维化)^[2]。

流行

全世界大约有500万人得这种病^[3]。
在美国大概有8万例肺纤维化病人^[1]。估计每年至少有3万新病例发现^[4]。
男性多于女性。

症状

呼吸急促
干咳
渐进性体重减少
疲劳或乏力
胸口不适，胸痛(偶尔)

诊断

美国胸腔学会与欧洲呼吸学会的专家小组提供了对特发性肺纤维化诊断的协商意见^[4]。首先排除其他有肺纤维化的疾病。一般认为只有靠肺活检才能确诊。

病史
肺部X光或CT扫描
肺功能测试
血氧含量
运动试验
支气管肺泡洗液 (BAL)
肺活检

療程選擇

療程選擇及早與正確診斷特發性肺纖維化是很重要，因為選擇療程如用藥、氧氣治療、治理咳嗽與肺部復康(包括特別運動方法或呼吸技巧)可幫助患者管理自身健康狀況和維持良好的生活質素。

預後 (預測疾病的可能結果)

被診斷患有特發性肺纖維化的預後因人而異,但約一半的患者會在確診後 3 至 4 年內死亡。^[5]

治疗

特發性肺纖維化患者的治療選擇十分有限。由於肺部結疤後不能回復到原來的狀況，現今唯一痊癒的方法是換肺手術，然而這種方法僅適用於極少數的患者。

醫學界為尋求醫治特發性肺纖維化，正進行多項的臨床實驗研究^[6]。期望尋找出一個既安全又有效，可改變病程，從而減慢特發性肺纖維化的疾病惡化。

近年，德國藥廠研發出新藥物「尼達尼布」，可減慢特發性肺纖維化的惡化速度，其療效突破醫學界的傳統局限^[7] 。美國食物及藥品管理局(FDA)早前已頒布優先檢討與突破性治療(Breakthrough Thearpy)法審程序^[8]^[9]，以加快批核「尼達尼布」的申請，希望能儘快幫助有需要的患者。於 2014 年 10 月，美國食物及藥品管理局(FDA)批准「尼達尼布」用於治療特發性肺纖維化(idiopathic pulmonary fibrosis, IPF)^[10]。在歐洲，「尼達尼布」早前亦被歐洲藥物管理局確認獲得特快審批，並於 2014 年 6 月被批准於歐盟地區上市，並以 Ofev 的商名作治療特發性肺纖維化的藥物推廣^[11]^[12]。

始於2008年，日本、欧洲、中国大陆相继批准了以吡非尼酮为API的药物治疗特发性肺纤维化。欧洲的Intermune公司的Esbriet，日本盐野义的Perispa，已及中国大陆北京康蒂尼药业的艾思瑞(Etuary)都已上市。美国新英格兰医学发表的三期临床数据显示，服用12个月后，具有延长无进展生存，延缓FVC下降，降低死亡风险的作用^[13]。
其他药剂治疗［如固醇类激素或抗癌药物(硫唑嘌呤或环磷酰胺)］，在最新的临床试验中发现不改善病人的生活品质，也不延长病人的生存时间。
氧气及呼吸輔助器具
肺移植，是另一个有效的治疗手段^[14]。
实验药物^[14]
- 干扰素伽玛(Interferon-gamma)^[15] ^[16]
- FG-3019（临床一期新抗体）

預後

預後差。 5年存活率42％或更低^[17]。

并发症

呼吸衰竭
慢性低氧血症(低血氧水平)
肺动脉高压
肺心病
红细胞增多

预防

避免吸烟，但是否有助于防止特发性肺纤维化，不得而知.

患者

著名評馬人董驃(卒於2006年2月22日)

参看

纤维化

参考文献

^ ^1.0 ^1.1 American Thoracic Society; European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Amer J Respir Crit Care Med. 2002 Jan 15;165(2):277-304.
^ Steele MP, Speer MC, Loyd JE, Brown KK, Herron A, Slifer SH, Burch LH, Wahidi MM, Phillips JA 3rd, Sporn TA, McAdams HP, Schwarz MI, Schwartz DA. Clinical and pathologic features of familial interstitial pneumonia. Am J Respir Crit Care Med. 2005 Nov 1;172(9):1146-5
^ Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis. 2008 Mar 26;3:8. Review. PubMed PMID: 18366757; PubMed Central PMCID: PMC2330030.>
^ ^4.0 ^4.1 American Thoracic Society (ATS), and the European Respiratory Society (ERS). American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Am J Respir Crit Care Med. 2000 Feb;161(2 Pt 1):646-64
^ Behr J., S2K-Leitlinie zur Diagnostik und Therapie der idiopathischen Lungenfibrose. Pneumologie Dtsch Arztebl Int 2013: 67: 81–111
^ Gomer R., et al. Investigational approaches to therapies for idiopathic pulmonary fibrosis. National Institutes of Health. 2010
^ Richeldi, L., et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. New England Journal of Medicine 2014 May; 370(22):2071-82. DOI: 10.1056/NEJMoa1402584.
^ FDA. CY 2014 CDER Breakthrough Therapy Calendar Year Approvals. Available at:http://www.fda.gov/downloads/regulatoryinformation/legislation/federalfooddrugandcosmeticactfdcact/significantamendmentstothefdcact/fdasia/ucm380724.pdf Accessed in Jun 2015.
^ FDA grants priority review for IPF drug application. Available at: http://www.modernmedicine.com/modern-medicine/content/fda-grants-priority-review-ipf-drug-application?page=full . Accessed in Jun 2015.
^ FDA. FDA approves Ofev to treat idiopathic pulmonary fibrosis. Available at:http://www.fda.gov/newsevents/newsroom/pressannouncements/ucm418994.htm . Accessed in Jun 2015.
^ ESMO. European Medicines Agency Recommends Granting a Marketing Authorisation for Nintedanib. Available at: http://www.esmo.org/Oncology-News/European-Medicines-Agency-Recommends-Granting-a-Marketing-Authorisation-for-Nintedanib. Accessed in Jun 2015.
^ Medscape. Nintedanib (Ofev) Gets EMA Panel Backing for IPF. Available at:http://www.medscape.com/viewarticle/835390. Accessed in Jun 2015.
^ King TE Jr1, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, Gorina E, Hopkins PM, Kardatzke D, Lancaster L, Lederer DJ, Nathan SD, Pereira CA, Sahn SA, Sussman R, Swigris JJ, Noble PW; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014 May 29;370(22):2083-92.PMID 24836312.
^ ^14.0 ^14.1 Walter N, Collard HR, King TE Jr. Current perspectives on the treatment of idiopathic pulmonary fibrosis. Proc Am Thorac Soc. 2006;3(4):330-338.
^ Raghu G, Brown KK, Bradford WZ, Starko K, Noble PW, Schwartz DA, King TE Jr; Idiopathic Pulmonary Fibrosis Study Group. A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. New England Journal of Medicine. 2004;350(2):125-133.
^ Andrew Pollack, Drug Maker Stops Work on Lung Disease Medicine, New York Times, March 6, 2007.
^ Jegal Y, Kim DS, Shim TS, Lim CM, Do Lee S, Koh Y, Kim WS, Kim WD, Lee JS, Travis WD, Kitaichi M, Colby TV. Physiology is a stronger predictor of survival than pathology in fibrotic interstitial pneumonia. Am J Respir Crit Care Med. 2005 Mar 15;171(6):639-44

外部链接

[ATS02-1] 1.0 ^1.1 American Thoracic Society; European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Amer J Respir Crit Care Med. 2002 Jan 15;165(2):277-304.

[2] Steele MP, Speer MC, Loyd JE, Brown KK, Herron A, Slifer SH, Burch LH, Wahidi MM, Phillips JA 3rd, Sporn TA, McAdams HP, Schwarz MI, Schwartz DA. Clinical and pathologic features of familial interstitial pneumonia. Am J Respir Crit Care Med. 2005 Nov 1;172(9):1146-5

[Orphanet-3] Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis. 2008 Mar 26;3:8. Review. PubMed PMID: 18366757; PubMed Central PMCID: PMC2330030.>

[ATS00-4] 4.0 ^4.1 American Thoracic Society (ATS), and the European Respiratory Society (ERS). American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Am J Respir Crit Care Med. 2000 Feb;161(2 Pt 1):646-64

[5] Behr J., S2K-Leitlinie zur Diagnostik und Therapie der idiopathischen Lungenfibrose. Pneumologie Dtsch Arztebl Int 2013: 67: 81–111

[6] Gomer R., et al. Investigational approaches to therapies for idiopathic pulmonary fibrosis. National Institutes of Health. 2010

[7] Richeldi, L., et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. New England Journal of Medicine 2014 May; 370(22):2071-82. DOI: 10.1056/NEJMoa1402584.

[8] FDA. CY 2014 CDER Breakthrough Therapy Calendar Year Approvals. Available at:http://www.fda.gov/downloads/regulatoryinformation/legislation/federalfooddrugandcosmeticactfdcact/significantamendmentstothefdcact/fdasia/ucm380724.pdf Accessed in Jun 2015.

[9] FDA grants priority review for IPF drug application. Available at: http://www.modernmedicine.com/modern-medicine/content/fda-grants-priority-review-ipf-drug-application?page=full . Accessed in Jun 2015.

[10] FDA. FDA approves Ofev to treat idiopathic pulmonary fibrosis. Available at:http://www.fda.gov/newsevents/newsroom/pressannouncements/ucm418994.htm . Accessed in Jun 2015.

[11] ESMO. European Medicines Agency Recommends Granting a Marketing Authorisation for Nintedanib. Available at: http://www.esmo.org/Oncology-News/European-Medicines-Agency-Recommends-Granting-a-Marketing-Authorisation-for-Nintedanib. Accessed in Jun 2015.

[12] Medscape. Nintedanib (Ofev) Gets EMA Panel Backing for IPF. Available at:http://www.medscape.com/viewarticle/835390. Accessed in Jun 2015.

[approved-13] King TE Jr1, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, Gorina E, Hopkins PM, Kardatzke D, Lancaster L, Lederer DJ, Nathan SD, Pereira CA, Sahn SA, Sussman R, Swigris JJ, Noble PW; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014 May 29;370(22):2083-92.PMID 24836312.

[treatment-14] 14.0 ^14.1 Walter N, Collard HR, King TE Jr. Current perspectives on the treatment of idiopathic pulmonary fibrosis. Proc Am Thorac Soc. 2006;3(4):330-338.

[15] Raghu G, Brown KK, Bradford WZ, Starko K, Noble PW, Schwartz DA, King TE Jr; Idiopathic Pulmonary Fibrosis Study Group. A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. New England Journal of Medicine. 2004;350(2):125-133.

[16] Andrew Pollack, Drug Maker Stops Work on Lung Disease Medicine, New York Times, March 6, 2007.

[17] Jegal Y, Kim DS, Shim TS, Lim CM, Do Lee S, Koh Y, Kim WS, Kim WD, Lee JS, Travis WD, Kitaichi M, Colby TV. Physiology is a stronger predictor of survival than pathology in fibrotic interstitial pneumonia. Am J Respir Crit Care Med. 2005 Mar 15;171(6):639-44

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@@ 第56行： / 第56行： @@
 醫學界為尋求醫治特發性肺纖維化，正進行多項的臨床實驗研究<ref> Gomer R., et al. Investigational approaches to therapies for idiopathic pulmonary fibrosis. National Institutes of Health. 2010</ref>。期望尋找出一個既安全又有效，可改變病程，從而減慢特發性肺纖維化的疾病惡化。
-* 近年，德國藥廠研發出新藥物[[「尼達尼布」]]，可減慢特發性肺纖維化的惡化速度，其療效突破醫學界的傳統局限<ref>Richeldi, L., et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. New England Journal of Medicine 2014 May; 370(22):2071-82. DOI: 10.1056/NEJMoa1402584.</ref> 。美國食物及藥品管理局(FDA)早前已頒布優先檢討與突破性治療(Breakthrough Thearpy)法審程序<ref>FDA. CY 2014 CDER Breakthrough Therapy Calendar Year Approvals. Available at:http://www.fda.gov/downloads/regulatoryinformation/legislation/federalfooddrugandcosmeticactfdcact/significantamendmentstothefdcact/fdasia/ucm380724.pdf Accessed in Jun 2015.</ref><ref>FDA grants priority review for IPF drug application. Available at: http://www.modernmedicine.com/modern-medicine/content/fda-grants-priority-review-ipf-drug-application?page=full . Accessed in Jun 2015.</ref>，以加快批核「[[尼達尼布]]」的申請，希望能儘快幫助有需要的患者。於 2014 年 10 月，美國食物及藥品管理局(FDA)批准「[[尼達尼布]]」用於治療特發性肺纖維化(idiopathic pulmonary fibrosis, IPF)<ref>FDA. FDA approves Ofev to treat idiopathic pulmonary fibrosis. Available at:http://www.fda.gov/newsevents/newsroom/pressannouncements/ucm418994.htm . Accessed in Jun 2015.</ref>。在歐洲，「尼達尼布」早前亦被歐洲藥物管理局確認獲得特快審批，並於 2014 年 6 月被批准於歐盟地區上市，並以 Ofev 的商名作治療特發性肺纖維化的藥物推廣<ref>ESMO. European Medicines Agency Recommends Granting a Marketing Authorisation for Nintedanib. Available at: http://www.esmo.org/Oncology-News/European-Medicines-Agency-Recommends-Granting-a-Marketing-Authorisation-for-Nintedanib. Accessed in Jun 2015.</ref><ref>Medscape. Nintedanib (Ofev) Gets EMA Panel Backing for IPF. Available at:http://www.medscape.com/viewarticle/835390. Accessed in Jun 2015.</ref>。
+* 近年，德國藥廠研發出新藥物「[[尼達尼布]]」，可減慢特發性肺纖維化的惡化速度，其療效突破醫學界的傳統局限<ref>Richeldi, L., et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. New England Journal of Medicine 2014 May; 370(22):2071-82. DOI: 10.1056/NEJMoa1402584.</ref> 。美國食物及藥品管理局(FDA)早前已頒布優先檢討與突破性治療(Breakthrough Thearpy)法審程序<ref>FDA. CY 2014 CDER Breakthrough Therapy Calendar Year Approvals. Available at:http://www.fda.gov/downloads/regulatoryinformation/legislation/federalfooddrugandcosmeticactfdcact/significantamendmentstothefdcact/fdasia/ucm380724.pdf Accessed in Jun 2015.</ref><ref>FDA grants priority review for IPF drug application. Available at: http://www.modernmedicine.com/modern-medicine/content/fda-grants-priority-review-ipf-drug-application?page=full . Accessed in Jun 2015.</ref>，以加快批核「[[尼達尼布]]」的申請，希望能儘快幫助有需要的患者。於 2014 年 10 月，美國食物及藥品管理局(FDA)批准「[[尼達尼布]]」用於治療特發性肺纖維化(idiopathic pulmonary fibrosis, IPF)<ref>FDA. FDA approves Ofev to treat idiopathic pulmonary fibrosis. Available at:http://www.fda.gov/newsevents/newsroom/pressannouncements/ucm418994.htm . Accessed in Jun 2015.</ref>。在歐洲，「尼達尼布」早前亦被歐洲藥物管理局確認獲得特快審批，並於 2014 年 6 月被批准於歐盟地區上市，並以 Ofev 的商名作治療特發性肺纖維化的藥物推廣<ref>ESMO. European Medicines Agency Recommends Granting a Marketing Authorisation for Nintedanib. Available at: http://www.esmo.org/Oncology-News/European-Medicines-Agency-Recommends-Granting-a-Marketing-Authorisation-for-Nintedanib. Accessed in Jun 2015.</ref><ref>Medscape. Nintedanib (Ofev) Gets EMA Panel Backing for IPF. Available at:http://www.medscape.com/viewarticle/835390. Accessed in Jun 2015.</ref>。
 *始於2008年，日本、欧洲、中国大陆相继批准了以吡非尼酮为API的药物治疗特发性肺纤维化。欧洲的Intermune公司的Esbriet，日本盐野义的Perispa，已及中国大陆北京康蒂尼药业的艾思瑞(Etuary)都已上市。美国新英格兰医学发表的三期临床数据显示，服用12个月后，具有延长无进展生存，延缓FVC下降，降低死亡风险的作用<ref name=approved>King TE Jr1, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, Gorina E, Hopkins PM, Kardatzke D, Lancaster L, Lederer DJ, Nathan SD, Pereira CA, Sahn SA, Sussman R, Swigris JJ, Noble PW; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014 May 29;370(22):2083-92.PMID 24836312.</ref>。