重症间质性肺病诊断与治疗课件.ppt

1、梅河口市新华医院梅河口市新华医院DPLD/ILDDPLD/ILD肉芽肿疾病肉芽肿疾病结节病、结节病、LCH胶原血管疾病胶原血管疾病肺肾综合征肺肾综合征吸入因素吸入因素职业（有机无机）职业（有机无机）环境（包括家居）环境（包括家居）气体蒸汽气溶胶气体蒸汽气溶胶爱好鸟宠物爱好鸟宠物遗传因素遗传因素家族性家族性IPF结节性硬化结节性硬化神经纤维瘤病神经纤维瘤病代谢储存病代谢储存病某些特异性疾病某些特异性疾病PAP癌性淋巴管炎癌性淋巴管炎LAM/IPH肺静脉阻塞性疾病肺静脉阻塞性疾病医源性（药物放射）医源性（药物放射）特发性间质性肺特发性间质性肺炎（炎（IIP）感染感染*病毒病毒非典型病原体非典型病原

2、体PCP继发于其他系统继发于其他系统疾病疾病 消化、肾消化、肾脏、血液等脏、血液等Adapted from Ryu JH,et al.Mayo Clin Proc.1998;73:1085-1101.Adapted from ATS/ERS.Am J Respir Crit Care Med.2002;165:277-304.1970s2003IdiopathicIPsHeterogeneousgroup that includeda number ofdiseasesUIP/IPFNSIPDIPRB-ILDAIPCellularFibroticInterstitial Lung Diseas

3、esSarcoidosisHypersensitivity PneumonitisAsbestosisLAMBMLLGCOPLIP特发性间质性肺炎（IIP）特发性肺纤维化（IPF/UIP）除 IPF 以外的特发性间质性肺炎呼吸性细支气管炎伴间质性肺病（RBILD）隐原性机化性肺炎（COP）脱屑型间质性肺炎（DIP）急性间质性肺炎（AIP）非特异性间质性肺炎（NSIP）淋巴细胞间质性肺炎（LIP）特发性间质性肺炎的分类特发性间质性肺炎的分类AIPARDSAIP05010015020025030045-5455-6465-7475+MaleFemale02040608010012045-5455-

4、6465-7475+MaleFemalePrevalenceIncidencePer 100,000Per 100,000 Incidence:30,000 patients/year Prevalence:80,000 current patients Age of onset:most 4070 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

5、.2006;174:810-816.UIP 时间和空间分布不一致 肌成纤维（纤维母）细胞灶 不同程度间质的炎症和胶原纤维 沉积指征指征方案方案PCPCMV1.AcuteP.EdemaPneumonia2.Pleural effusion1.pulmonary edema2.lymphangitic carcinomatosis3.lymphoma4.collagen vascular disease3.PneumothoraxlymphangioleiomyomatosisLCH4.Predominantly Below with reduced volume1.Asbestosis2.Asp

6、iration(chronic)3.Pulmonary fibrosis(idiopathic)4.Collagen vascular disease鉴别诊断需要参考的其他方面鉴别诊断需要参考的其他方面5.A middle or upper lung predominant1.Mycobacterial or fungal disease2.Silicosis3.Sarcoidosis4.Langerhans Cell Histiocytosis6.Associated lymphadenopathy1.Sarcoidosis2.neoplasm(lymphangitic carcinomatosis,lymphoma,metastases)3.infection(viral,mycobacterial,or fungal)4.silicosis7.Pleural Thickening and or CalcificationAsbestosis鉴别诊断需要参考的其他方面鉴别诊断需要参考的其他方面二一五年九月二十三日二一五年九月二十三日