特发性间质性肺炎徐作军PPT.pptx

特发性间质性肺炎Idiopathic Interstitial Pneumonitis北京协和医院 呼吸内科徐作军1、Major Idiopathic Interstitial PneumoniasChronic Fibrosing IIPsIdiopathic pulmonary fibrosis（UIP）Idiopathic nonspecific interstitial pneumonia（NSIP）Smoking-related IIPsRespiratory bronchiolitis interstitial lung disease（RBILD）Desquamative interstitial pneumonia（DIP）Acute or Subacute IIPsAcute interstitial pneumonia（AIP）Cryptogenic organizing pneumonia（COP）?2、Rare Idiopathic Interstitial PneumoniasIdiopathic lymphoid interstitial pneumonia（iLIP）Idiopathic pleuroparenchymal fibroelastosis（iPPFE）3、Unclassifiable idiopathic interstitial pneumoniasRare histologic patterns: AFOP、BCIP特发性间质性肺炎分类 (2013) 特发性间质性肺炎的分类(2013年)特发性间质性肺炎（IIP）少见的IIP主要的IIP不能分类的IIP特发性淋巴细胞间质性肺炎 （iLIP）特发性胸膜肺弹力纤维增生症 （iPPFE）家族性 2-20%, 非家族性 80%由于证据不够下列二个病理类型未被列入疾病分类:急性纤维素性机化性肺炎气道中心性间质性肺炎慢性致纤维化性IP IPF NSIP急性/亚急性IP COP AIP吸烟相关性IP DIP RBILD特发性肺纤维化（IPF）定义2011年ATS/ERS/JRS/ALAT IPF指南定义：慢性、进行性致纤维化的间质性肺炎，局限于肺部原因不明、主要出现在老年患者表现为劳力性呼吸困难，进行性加重，肺功能恶化，预后差组织病理学和/或影像学HRCT表现为普通型间质性肺炎(UIP)Am J Respir Cri Care Med.,2011,187,788-824 IncidencePrevalence120300100250MaleMaleFemaleFemale80200Per 100,000Per 100,000601504010020500045-5455-6465-7475+45-5455-6465-7475+ Incidence: 30,000 patients/year Prevalence: 80,000 current patients Age of onset: most 40–70 years Two-thirds 60 years old at presentation Males femalesATS/ERS. Am J Respir Crit Care Med. 2000;161:646-664.Raghu G, et al. Am J Respir Crit Care Med. 2006;174:810-816.美国流行病学资料IPF/UIPAm J Respir Cri Care Med.,2011,187,788-824 IPF临床表现成人不能解释的劳累型呼吸困难咳嗽双肺底吸气爆裂音（Velcro）杵状指Subjected to external reviewIPF诊断标准排除其它已知原因导致的ILD (如：环境和职业导致的肺病，CTD-ILD，和药物性肺病等)。在没有外科肺活检资料条件下，胸部HRCT呈现典型的UIP表现。有外科肺活检资料条件下，胸部HRCT和病理均符合UIP表现。Subjected to external reviewDiagnostic algorithm for IPFPossible UIPInconsistent with UIPUIP / Probable / Possible UIPNon-classifiable fibrosisSubjected to external reviewHRCT criteria for UIP pattern 典型UIP (all four features) 可能UIP (all three features) 不符合UIP (any of the seven features)胸膜下, 基底部为主网格状阴影蜂窝伴有或不伴