特发性肺纤维化

通常与胶原血管病有关；因此，患者可能有皮肤、关节或其他全身性表现。

胸部CT有更多的磨玻璃影，无蜂窝肺。

当继发于潜在的结缔组织病时，可见自身抗体阳性。[54]American Thoracic Society (ATS), European Respiratory Society (ERS). 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.http://www.atsjournals.org/doi/full/10.1164/ajrccm.165.2.ats01#.UmEU8tglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/11790668?tool=bestpractice.com[55]Travis WD, Costabel U, Hansell DM, et al; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733-748.http://www.atsjournals.org/doi/full/10.1164/rccm.201308-1483ST#.VEpLaBbgV9Mhttp://www.ncbi.nlm.nih.gov/pubmed/24032382?tool=bestpractice.com

既往被称为闭塞性细支气管炎伴机化性肺炎或BOOP；通常与以往的感染或胶原血管病相关。

全身性表现包括消瘦、发热、关节症状和皮疹较为常见。[56]Cordier JF. Cryptogenic organising pneumonia. Eur Respir J. 2006;28:422-446.http://erj.ersjournals.com/cgi/content/full/28/2/422http://www.ncbi.nlm.nih.gov/pubmed/16880372?tool=bestpractice.com

胸部CT可见斑片状实变、孤立性结节或浸润性网格影。[56]Cordier JF. Cryptogenic organising pneumonia. Eur Respir J. 2006;28:422-446.http://erj.ersjournals.com/cgi/content/full/28/2/422http://www.ncbi.nlm.nih.gov/pubmed/16880372?tool=bestpractice.com

在既往体健者发病，数周前通常有类流感样的前驱症状。

AIP较为少见，表现为突然恶化的低氧血症和呼吸衰竭。[53]Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am J Respir Crit Care Med. 1998;157:1301-1315.http://www.atsjournals.org/doi/full/10.1164/ajrccm.157.4.9707039#.UmEVMdglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/9563754?tool=bestpractice.com

胸部影像表现为弥漫性双侧磨玻璃影或肺泡填充型。[54]American Thoracic Society (ATS), European Respiratory Society (ERS). 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.http://www.atsjournals.org/doi/full/10.1164/ajrccm.165.2.ats01#.UmEU8tglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/11790668?tool=bestpractice.com[55]Travis WD, Costabel U, Hansell DM, et al; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733-748.http://www.atsjournals.org/doi/full/10.1164/rccm.201308-1483ST#.VEpLaBbgV9Mhttp://www.ncbi.nlm.nih.gov/pubmed/24032382?tool=bestpractice.com

呼吸道症状相似。

吸烟人群患病。[53]Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am J Respir Crit Care Med. 1998;157:1301-1315.http://www.atsjournals.org/doi/full/10.1164/ajrccm.157.4.9707039#.UmEVMdglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/9563754?tool=bestpractice.com

胸部CT可见弥漫性小结节影或网状病变。[54]American Thoracic Society (ATS), European Respiratory Society (ERS). 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.http://www.atsjournals.org/doi/full/10.1164/ajrccm.165.2.ats01#.UmEU8tglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/11790668?tool=bestpractice.com[55]Travis WD, Costabel U, Hansell DM, et al; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733-748.http://www.atsjournals.org/doi/full/10.1164/rccm.201308-1483ST#.VEpLaBbgV9Mhttp://www.ncbi.nlm.nih.gov/pubmed/24032382?tool=bestpractice.com

肺功能检查提示阻塞性和限制性功能障碍。[1]American Thoracic Society; European Respiratory Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Am J Respir Crit Care Med. 2000;161:646-664.http://www.atsjournals.org/doi/full/10.1164/ajrccm.161.2.ats3-00#.UmEPiNglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/10673212?tool=bestpractice.com

罕见，吸烟者罹患，亚急性病程，无疤痕性肺纤维化。[1]American Thoracic Society; European Respiratory Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Am J Respir Crit Care Med. 2000;161:646-664.http://www.atsjournals.org/doi/full/10.1164/ajrccm.161.2.ats3-00#.UmEPiNglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/10673212?tool=bestpractice.com

CT可见弥漫性磨玻璃影。

肺功能检查提示限制性功能障碍。[54]American Thoracic Society (ATS), European Respiratory Society (ERS). 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.http://www.atsjournals.org/doi/full/10.1164/ajrccm.165.2.ats01#.UmEU8tglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/11790668?tool=bestpractice.com[55]Travis WD, Costabel U, Hansell DM, et al; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733-748.http://www.atsjournals.org/doi/full/10.1164/rccm.201308-1483ST#.VEpLaBbgV9Mhttp://www.ncbi.nlm.nih.gov/pubmed/24032382?tool=bestpractice.com

经常继发于干燥综合征、艾滋病或蛋白异常血症，所以患者可能有这些疾病的症状或体征。

胸部CT可能出现结节、磨玻璃影、网格影和薄壁囊腔影。[54]American Thoracic Society (ATS), European Respiratory Society (ERS). 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.http://www.atsjournals.org/doi/full/10.1164/ajrccm.165.2.ats01#.UmEU8tglgZkhttp://www.ncbi.nlm.nih.gov/pubmed/11790668?tool=bestpractice.com[55]Travis WD, Costabel U, Hansell DM, et al; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733-748.http://www.atsjournals.org/doi/full/10.1164/rccm.201308-1483ST#.VEpLaBbgV9Mhttp://www.ncbi.nlm.nih.gov/pubmed/24032382?tool=bestpractice.com

出现皮肤、关节或其他血清学异常。[57]Chan-Yeung M, Muller NL. Cryptogenic fibrosing alveolitis. Lancet. 1997;350:651-656.http://www.ncbi.nlm.nih.gov/pubmed/9288060?tool=bestpractice.com

可能出现皮疹。

不典型的胸部CT表现，呈非外周分布。

出现结缔组织相关的抗体。

呼吸道症状与IPF相似，与暴露于可能导致肺纤维化的药物有关，如胺碘酮、呋喃妥因和博来霉素。

胸部CT和肺功能均为非特异的。

具有石棉接触史。

胸膜疾病的体格检查通常正常，除非患有弥漫性胸膜增厚或良性胸腔积液。在患有这些疾病时，听诊呼吸音减低，叩诊呈浊音。

胸部影像可见胸膜斑；活检可见石棉小体。[57]Chan-Yeung M, Muller NL. Cryptogenic fibrosing alveolitis. Lancet. 1997;350:651-656.http://www.ncbi.nlm.nih.gov/pubmed/9288060?tool=bestpractice.com

呼吸道症状通常与致病性抗原急性暴露有关，如青贮饲料或干草中的细菌、霉菌。

血液中查见致病性抗原的IgG抗体，HRCT可见磨玻璃影，肺活检示肉芽肿。

肺外受累

与IPF相比，患者较为年轻。

胸部影像中淋巴结肿大更为明显。[57]Chan-Yeung M, Muller NL. Cryptogenic fibrosing alveolitis. Lancet. 1997;350:651-656.http://www.ncbi.nlm.nih.gov/pubmed/9288060?tool=bestpractice.com

罕见疾病。

吸烟史与自发性气胸病史。

胸部CT示上和中肺野分布的囊腔病变伴结节。

罕见疾病。

绝经前女性患病；自发性气胸常见。

全肺薄壁囊性病变；肺功能检查提示阻塞性和限制性障碍。