美国胸科协会(ATS)和欧洲呼吸学会(ERS)联合发表的共识中，将特发性肺纤维化(IPF)定义为原因不明并以普通型间质性肺炎(UIP)为特征性病理改变的一种慢性纤维化性间质性肺疾病。在2000年ATS／ERS的IPF共识意见 及2003年中华医学会呼吸病学分会IPF的诊断和治疗指南(草案) 中均推荐的治疗方案为糖皮质激素，或与细胞毒制剂(环磷酰胺及硫唑嘌呤)联合使用。但目前尚缺乏循证医学证据支持该治疗方案能够提高IPF患者生活质量或生存率 。近年来随着对IPF的发病机制认识的深入，越来越多的临床医师和研究者对IPF患者是否需要用糖皮质激素等药物的治疗提出了质疑。
Citation：
CAI Hourong. Idiopathic pulmonary fibrosis：acting or waiting？. Chinese Journal of Respiratory and Critical Care Medicine, 2008, 08(5): 321-322. doi:
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Demedts M,Costabel U．ATS／ERS international multidiscipIinary consensus classification of the idiopathic interstitial pneumonias．Eur Respir J，2002，19：794-796．.
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American Thoracic Society．Idiopathic pulmonary fibrosis：diagnosis and treatment．International consensus statement．American Thoracic Society(ATS)，and the European Respiratory Society (ERS)．Am J Respir Crit Care Med.2000，161：646-664．.
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中华医学会呼吸病学分会．特发f生肺(间质)纤维化诊断和治疗指南(草案)．中华结核和呼吸杂志，2002，25：387-389．.
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Raghu G．Idiopathic pulmonary fibrosis：treatment options in pursuit of evidence-based approaches．Eur Respir J，2006，28：463-465．.
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Lynch JP 3rd，White E，Flaherty K．Corticosteroids in idiopathic pulmonary fibrosis．Curr Opin Pulm Med，2001，7：298-308．.
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Swigris JJ，Kuschner WG，Kelsey JL，et al．Idiopathic pulmonary fibrosis：challenges and opportunities for the clini cian and investigator．Chest，2005，127：275—283．.
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| 7. |
Sjoraker JA，Ryu JH，Edwin MK，et al．Prognostic significanoe of histopathologic subsets in idiopathic pulmonary fibrosis．Am J Respir Crit Care Med，1998，157：199-203．.
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| 8. |
Daniels CE，Ryu JH．Treatment of idiopathic pulmonary fibmsis．Semin Respir Crit Care Med，2006，27：668-676．.
|
| 9. |
Collard HR，Ryu JH，Douglas WW ，et al．Combined cortico-steroid and cyclophosphamide therapy does not alter Survival in idiopathic pulmonary fibrosis．Chest，2004，125：2169-2174.
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| 10. |
Raghu G，Depaso WJ，Cain K．Azathiopfine combined with prednisone in the treatment of idiopathic pulmonary fibrosis：a prospective double-blind，randomized，placebo-controlled clinical tria1．Am Rev Respir Dis，1991，144：291-296．.
|
- 1. Demedts M,Costabel U．ATS／ERS international multidiscipIinary consensus classification of the idiopathic interstitial pneumonias．Eur Respir J，2002，19：794-796．.
- 2. American Thoracic Society．Idiopathic pulmonary fibrosis：diagnosis and treatment．International consensus statement．American Thoracic Society(ATS)，and the European Respiratory Society (ERS)．Am J Respir Crit Care Med.2000，161：646-664．.
- 3. 中华医学会呼吸病学分会．特发f生肺(间质)纤维化诊断和治疗指南(草案)．中华结核和呼吸杂志，2002，25：387-389．.
- 4. Raghu G．Idiopathic pulmonary fibrosis：treatment options in pursuit of evidence-based approaches．Eur Respir J，2006，28：463-465．.
- 5. Lynch JP 3rd，White E，Flaherty K．Corticosteroids in idiopathic pulmonary fibrosis．Curr Opin Pulm Med，2001，7：298-308．.
- 6. Swigris JJ，Kuschner WG，Kelsey JL，et al．Idiopathic pulmonary fibrosis：challenges and opportunities for the clini cian and investigator．Chest，2005，127：275—283．.
- 7. Sjoraker JA，Ryu JH，Edwin MK，et al．Prognostic significanoe of histopathologic subsets in idiopathic pulmonary fibrosis．Am J Respir Crit Care Med，1998，157：199-203．.
- 8. Daniels CE，Ryu JH．Treatment of idiopathic pulmonary fibmsis．Semin Respir Crit Care Med，2006，27：668-676．.
- 9. Collard HR，Ryu JH，Douglas WW ，et al．Combined cortico-steroid and cyclophosphamide therapy does not alter Survival in idiopathic pulmonary fibrosis．Chest，2004，125：2169-2174.
- 10. Raghu G，Depaso WJ，Cain K．Azathiopfine combined with prednisone in the treatment of idiopathic pulmonary fibrosis：a prospective double-blind，randomized，placebo-controlled clinical tria1．Am Rev Respir Dis，1991，144：291-296．.
