目的 探討特發(fā)性肺纖維化( IPF) 急性加重患者的臨床和胸部影像學特點, 提高對IPF急性加重的認識。方法 回顧性分析2006 年4 月至2008 年7 月南京鼓樓醫(yī)院呼吸科收治的2 例 IPF 急性加重患者的臨床和影像學資料, 并復習相關(guān)文獻。結(jié)果 2 例患者均為老年男性, 主要的癥狀和體征為氣短、咳嗽及吸氣性爆裂音。呼吸困難分別在1 周和半月內(nèi)加重; 2 例患者氧合指數(shù)均小于225 mmHg; 急性加重時2 例患者HRCT 表現(xiàn)為兩下肺分布的網(wǎng)狀影、蜂窩影、牽拉性細支氣管擴張和支氣管擴張等典型的IPF 表現(xiàn), 并出現(xiàn)新的磨玻璃影。其中1 例患者的雙肺新出現(xiàn)的磨玻璃影,沿胸膜下蜂窩肺外周分布; 另1 例患者HRCT 表現(xiàn)為新出現(xiàn)的磨玻璃影呈彌漫性分布。2 例患者均接受激素治療。1 例患者氣緊、咳嗽癥狀明顯緩解, 胸部HRCT 的磨玻璃樣影基本吸收; 另1 例患者死于呼吸衰竭。結(jié)論 少數(shù)IPF 患者可在無誘因下出現(xiàn)急性加重。IPF 急性加重患者主要臨床表現(xiàn)為呼吸困難癥狀在短期內(nèi)急劇惡化, 其胸部影像學特點為在典型的IPF 表現(xiàn)基礎上出現(xiàn)新的磨玻璃影。
引用本文： 代靜泓,苗立云,曹敏,蔡后榮. 特發(fā)性肺纖維化急性加重的臨床和影像學特點( 二例報道及文獻復習). 中國呼吸與危重監(jiān)護雜志, 2009, 09(4): 355-359. doi: 復制
| 1. | American Thoracic Society/ European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med, 2002 , 165 :277-304. |
| 2. | Collard HR, Moore BB, Flaherty KR, et al. Acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med, 2007 ,176: 636-643. |
| 3. | Kondoh Y, Taniguchi H, Kawabata Y, et al. Acute exacerbation in idiopathic pulmonary fibrosis: analysis of clinical and pathologic findings in three cases. Chest, 1993, 103: 1808-1812. |
| 4. | 代靜泓, 蔡后榮. 特發(fā)性肺纖維化急性加重的研究進展. 中國呼吸與危重監(jiān)護雜志, 2009, 8: 87-90. |
| 5. | Kim DS, Park JH, Park BK, et al. Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J,2006, 27: 143-150. |
| 6. | 于娜, 劉知陶, 姜莉, 等. 特發(fā)性肺纖維化急性加重1 例并文獻復習. 中國實用內(nèi)科雜志, 2006, 26: 986-988. |
| 7. | Akira M, Hamada H, Sakatani M, et al. CT findings during phase of accelerated deterioration in patients with idiopathic pulmonary fibrosis. Am J Roentgenol, 1997, 168: 79-83. |
| 8. | Akira M, Kozuka T, Yamamoto S, et al. Computed tomography findings in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med, 2008, 178: 372-378. |
| 9. | Ambrosini V, Cancellieri A, Chilosi M, et al. Acute exacerbation of idiopathic pulmonary fibrosis: report of a series. Eur Respir J,2003, 22: 821-826. |
| 10. | Yokoyama A, Kohno N, Hamada H, et al. Circulating KL-6 predicts the outcome of rapidly progressive idiopathic pulmonary fibrosis.Am J Respir Crit Care Med, 1998, 158: 1680-1684. |
| 11. | Martinez FJ, Safrin S, Weycker D, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med, 2005 ,142: 963-967. |
| 12. | 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: 1040-1047. |
| 13. | Parambil JG, Myers JL, Ryu JH. Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy. Chest, 2005, 128: 3310 -3315. |
| 14. | Yüksel M, Ozyurtkan MO, Bostanci K, et al Acute exacerbation of interstitial fibrosis after pulmonary resection. Ann Thorac Surg,2006, 82: 336-338. |
| 15. | Suga T, Sugiyama Y, Ohno S, et al. Two cases of idiopathic interstitial pneumonia which developed acute exacerbation after bronchoalveolar lavage. Nihon Kyobu Shikkan Gakkai Zasshi,1994, 32: 174-178. |
- 1. American Thoracic Society/ European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med, 2002 , 165 :277-304.
- 2. Collard HR, Moore BB, Flaherty KR, et al. Acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med, 2007 ,176: 636-643.
- 3. Kondoh Y, Taniguchi H, Kawabata Y, et al. Acute exacerbation in idiopathic pulmonary fibrosis: analysis of clinical and pathologic findings in three cases. Chest, 1993, 103: 1808-1812.
- 4. 代靜泓, 蔡后榮. 特發(fā)性肺纖維化急性加重的研究進展. 中國呼吸與危重監(jiān)護雜志, 2009, 8: 87-90.
- 5. Kim DS, Park JH, Park BK, et al. Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J,2006, 27: 143-150.
- 6. 于娜, 劉知陶, 姜莉, 等. 特發(fā)性肺纖維化急性加重1 例并文獻復習. 中國實用內(nèi)科雜志, 2006, 26: 986-988.
- 7. Akira M, Hamada H, Sakatani M, et al. CT findings during phase of accelerated deterioration in patients with idiopathic pulmonary fibrosis. Am J Roentgenol, 1997, 168: 79-83.
- 8. Akira M, Kozuka T, Yamamoto S, et al. Computed tomography findings in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med, 2008, 178: 372-378.
- 9. Ambrosini V, Cancellieri A, Chilosi M, et al. Acute exacerbation of idiopathic pulmonary fibrosis: report of a series. Eur Respir J,2003, 22: 821-826.
- 10. Yokoyama A, Kohno N, Hamada H, et al. Circulating KL-6 predicts the outcome of rapidly progressive idiopathic pulmonary fibrosis.Am J Respir Crit Care Med, 1998, 158: 1680-1684.
- 11. Martinez FJ, Safrin S, Weycker D, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med, 2005 ,142: 963-967.
- 12. 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: 1040-1047.
- 13. Parambil JG, Myers JL, Ryu JH. Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy. Chest, 2005, 128: 3310 -3315.
- 14. Yüksel M, Ozyurtkan MO, Bostanci K, et al Acute exacerbation of interstitial fibrosis after pulmonary resection. Ann Thorac Surg,2006, 82: 336-338.
- 15. Suga T, Sugiyama Y, Ohno S, et al. Two cases of idiopathic interstitial pneumonia which developed acute exacerbation after bronchoalveolar lavage. Nihon Kyobu Shikkan Gakkai Zasshi,1994, 32: 174-178.