ILD概论2010-3-18.pptVIP

* Cryptogenic organising pneumonia patchy consolidations (95%) perilobular opacity (50%) Ujita, Radiology 2004; 232: 757-61 * Congestion oedema Exudative phase Acute interstitial pneumonia, AIP acute onset, with systemic features: idiopathic ARDS granulocytes + occasional lymphocytes; debris survival from diagnosis often days despite mechanical support * Desquamative interstitial pneumonia, DIP smoker BAL: AM+++N+E+L * Respiratory bronchiolitis- interstitial lung disease cigarette smoker obstructive or restrictive lung function AM with smoker’s inclusions on BAL * Lymphocytic interstitial pneumonia, LIP AIDS lymphoproliferative rheumatological idiopathic (rare) lymphocytes on BAL * 已知原因DPLD drugs or CTD IIP 肉芽肿性 DPLD 其它DPLD LAM, HX etc. Diffuse Parenchymal Lung Disease IIP(非IPF） IPF DIP AIP LIP NSIP COP RB-ILD * Radiologists Pathologists Clinicians (Pulmonologists) History Exposure，Drugs Symptoms Signs Systemic Diseases (CVD) Age Should know, identify and report pattern-specific features Should know, identify and report pattern-specific features Correct IIP diagnoses need teamwork and experience Flaherty KR, AJRCCM 2004;170:904-10 * Ideal ILD doctor Radiologist Pathologist Pulmonologist * 间质性肺疾病的概念与分类 间质性肺疾病的诊断流程 间质性肺疾病的治疗原则 * 1.抗炎制剂 糖皮质激素 硫唑嘌呤/环磷酰胺 2.抗氧化制剂 N-乙酰半胱胺酸（NAC) 3.抗纤维化制剂 IFN-? 吡非尼酮Pirfenidone 4.抗凝治疗 5.内皮受体拮抗剂 Bosentan 6.纤维原性生长因子抑制剂 BIBF1199（抑制PDGFR,FGFR,VEGFR) 7.抗TNF-a：TNF-a拮抗剂 …… 药物治疗探索与评价 * 综合治疗 病因治疗 防控感染 防治静脉血栓栓塞症 肺康复治疗、运动训炼、教育、心理治疗 氧疗：对有严重低氧血症者，应予氧疗 肺动脉高压治疗 肺移植（有适应证者） 肺移植是唯一能够延长进展期IPF生存率的治疗 1/3 IPF患者在等待肺移植时死亡 * 外源性过敏性肺泡炎 尘肺或矽肺（焊工肺） 弥漫性肺泡出血 肺泡微石症 肺水肿等 …… 实质性结节：常见疾病 * 小叶中心性结节 Centrilobular nodule DPB: CT shows extensive centrilobular nobules, by their short distance from the pleural surface and their connection to more central branching structures. EAA: HRCT shows profuse, poorly defined ground-glass nodules typical for