新指南北京康健ppt课件

ATS/ERS/JRS/ALAT 特发性肺纤维化新循证指南解读 中国医科大学呼吸疾病研究所 康 健 Methodology: Question-based Approach Literature Review (MEDLINE search 1996-2006, and further update) Evidence Profiles (GRADE criteria, Schunemann AJRCCM 2006) Quality of Evidence (high, moderate, low, or very low) Strength of Recommendations (strong or weak) by vote Idiopathic Pulmonary Fibrosis: Evidence Based Guidelines IPF的定义 IPF是一种特殊形式的病因未明的慢性进展性致 纤维化性间质性肺炎，主要在老年发病，病变局限于 肺部，组织病理学和/或影像学表现具有寻常型间质性 肺炎的特征。诊断IPF需要排除其他各种间质性炎， 包括其他类型的特发性间质性肺炎（IIP）和与环境暴 露、药物或系统性疾病相关的间质性肺疾病。 IPF的危险因素 lAge lCigarette Smoking OR 1.6 2.9 lGastroesophageal Reflux lEnvironmental Factors metal dust wood dust lInfectious Agents viruses: EBV, herpes viruses lGenetic Predisposition no good predisposing genotypes? Major Criteria (presence of all) Exclusion of other known causes of ILD evidence of restriction and impaired gas exchange Typical HRCT features Transbronchial lung biopsy or BAL showing no features to support an alternative diagnosis Minor Criteria (at least 3 of 4) Age 50 yr Insidious onset of otherwise unexplained dyspnea on exertion Duration of illness 3 mo Bibasilar, inspiratory crackles (dry or “Velcro”-type in quality) ATS/ERS CRITERIA FOR DIAGNOSIS OF IDIOPATHIC PULMONARY FIBROSIS IN ABSENCE OF SURGICAL LUNG BIOPSY ATS/ERS criteria for diagnosis of IPF in absence of surgical lung biopsy (2000) Am J Respir Crit Care Med 2000; 161:646-664 IPF的诊断 除外已知原因引起的ILD（如环境暴露、结缔组织 病、药物性等） 无外科性肺活检者：HRCT表现为UIP型 进行外科性肺活检者：结合HRCT和组织病理学所见 IPF诊断流程 Possible UIP Inconsistent with UIP UIP / Probable / Possible UIP Non-classifiable fibrosis HRCT诊断UIP的条件 UIP 型 (符合4项项全部) 可能UIP 型 (符合3项项全部) 不符合 UIP 型 ( 7 项项中任何之一 ) 1. 病变主要位于胸膜下 和肺 基底部 2. 异常的网格状阴影 3. 蜂窝样改变，伴或不伴 牵 拉性支气管扩张 4. 无不符合UIP型的任何一条 （见不符合UIP型栏） 1.病变主要位于胸膜下和肺 基底部 2.异常的网格状阴影 3.无不符合UIP型的任何一条 （见不符合UIP型栏） 1. 病变主要分布于上、中肺野 2. 病变主要沿支气管血管束分布 3. 广泛磨玻璃样影（范围超过网 格样影） 4. 大量微结节影（双侧，上肺分 布为主） 5. 散在囊状病变（多发，双侧， 远离蜂窝肺区域） 6. 弥漫性马赛克征/气体陷闭（双 侧，3个及肺叶受累） 7. 支气管肺段/肺叶实变 组织病理学诊断 UIP型的条件 Marked fibrosis +/- honeycombing Fibroblastic foci Patchy involvement Subpleural, paraseptal distribution Absence of features against a diagnosis of UIP 组织病理学诊断 UIP all criteria present Probable UIP all but absence of either patchy involvement or fibroblastic foci or honeycombing only (end-stage fibrotic lung) Possible UIP patchy or diffuse lung fibrosis and absence of other criteria for UIP Not UIP (any of the six) hyaline membranes organizing pneumonia granulomas marked interstitial inflammation away from honeycombing predominant airway centered changes other features suggestive of an alternate diagnosis 透明膜 机化性肺炎 肉芽肿 远离蜂窝区有明显的炎性细胞浸润 病变以气道为中心分布 其他提示另一种诊断的特征 HRCT结合组织病理学对IPF的诊断 HRCT Pattern Surgical Lung Biopsy Pattern (when performed) Diagnosis of IPF UIP型 UIP YES Probable UIP Possible UIP Non-classifiable fibrosis Not UIPNo HRCT结合组织病理学对IPF的诊断 HRCT Pattern Surgical Lung Biopsy Pattern (when performed) Diagnosis of IPF 可能 UIP型 UIP YES Probable UIP Possible UIP Probable Non-classifiable fibrosis Not UIPNo Multidisciplinary discussion: sampling error on SLB? adequate HRCT? HRCT Pattern Surgical Lung Biopsy Pattern (when performed) Diagnosis of IPF 不符合 UIP型 UIPPossible Probable UIP No Possible UIP Non-classifiable fibrosis Not UIP HRCT结合组织病理学对IPF的诊断 Multidisciplinary discussion: sampling error on SLB? adequate HRCT? IPF的药物治疗 Strong recommendation against the use: Corticosteroid mono therapy (very low) Colchicine (very low) Cyclosporine A (very low) Combined corticosteroid and immune-modulator therapy (low) Interferon gamma 1b (high) Bosentan (moderate) Etanercept (moderate) Weak recommendation against the use: Combined acetylcysteine + aza + pred (low) Acetylcysteine monotherapy (low) Anticoagulation (very low) Pirfenidone (low to moderate) IPF的药物治疗 Implications of a weak recommendation against: Patients: The majority of people in this situation would not want the above treatment, but a minority would. Clinicians: Be more prepared to help patients to make a decision that is consistent with the patients own values. Policy makers: There is a need for substantial debate and involvement of stakeholders. BTS ILD guideline 2008: Weak recommendation IPF的其它治疗 Strong recommendation for the use of: - LTOT in clinically significant resting hypoxemia (very low) - Lung transplantation (very low) Weak recommendation for the use of: - Pulmonary rehabilitation (low) - Corticosteroids in acute exacerbation (very low) - Therapy for asymptomatic gastroesoph. reflux (very low) Weak recommendation against the use: - Specific pulmonary hypertension therapy (very low) - Mechanical ventilation for respiratory failure (low) IPF的治疗策略 TIME Diagnos is of IPF DISEASE PROGRESSION Monitor every 4-6 months or sooner as clinically indicated ACUTE EXACERBATION Corticosteroids RESPIRATORY FAILURE (due to progression of IPF) No mechanical ventilation Evaluate and list for lung transplantati on Patients should be made aware of available clinical trials for possible enrollment at all stages TREATMENT CONSIDERATIONS NON PHARMACOLOGICAL Oxygen supplementation Pulmonary rehabilitation PHARMACOLOGICAL Di