特發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)_《中國呼吸與危重監護雜志》

作者：

代靜泓 , 苗立云 , 曹敏 ,  蔡后榮

南京大學醫學院附屬鼓樓醫院呼吸內科（江蘇南京 210008）;

關鍵詞：

特發性肺纖維化急性加重癥狀體征影像診斷高分辨率CT

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目的探討特發性肺纖維化( 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, 并復習相關文獻。結果 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 周和半月內加重; 2 例患者氧合指數均小于225 mmHg; 急性加重時2 例患者HRCT 表現為兩下肺分布的網狀影、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, 并出現新的磨玻璃影。其中1 例患者的雙肺新出現的磨玻璃影,沿胸膜下蜂窩肺外周分布; 另1 例患者HRCT 表現為新出現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。結論少數IPF 患者可在無誘因下出現急性加重。IPF 急性加重患者主要臨床表現為呼吸困難癥狀在短期內急劇惡化, 其胸部影像學特點為在典型的IPF 表現基礎上出現新的磨玻璃影。

引用本文： 代靜泓,苗立云,曹敏,蔡后榮. 特發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. 特發性肺纖維化急性加重的研究進展. 中國呼吸與危重監護雜志, 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. 特發性肺纖維化急性加重1 例并文獻復習. 中國實用內科雜志, 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. 特發性肺纖維化急性加重的研究進展. 中國呼吸與危重監護雜志, 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. 特發性肺纖維化急性加重1 例并文獻復習. 中國實用內科雜志, 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.

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