IPF新指南北京-康健

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,AgeCigarette SmokingOR 1.6 2.9Gastroesophageal RefluxEnvironmental Factorsmetal dustwood dustInfectious Agentsviruses: EBV, herpes virusesGenetic Predispositionno good predisposing genotypes?,ATS/ERS CRITERIA FOR DIAGNOSIS OF IDIOPATHICPULMONARY 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,HRCT诊断UIP的条件,组织病理学诊断 UIP型的条件,Marked fibrosis +/- honeycombingFibroblastic foci Patchy involvementSubpleural, paraseptal distributionAbsence of features against a diagnosis of UIP,组织病理学诊断,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结合组织病理学对IPF的诊断, Multidisciplinary discussion: sampling error on SLB? adequate HRCT?,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,Diagnosis of IPF,DISEASE PROGRESSIONMonitor every 4-6 months or sooner as clinically indicatedACUTE EXACERBATIONCorticosteroidsRESPIRATORY FAILURE(due to progression of IPF)No mechanical ventilation,Evaluate and list for lung transplantation,Patients should be made aware of available clinical trials for possible enrollment at all stages,TREATMENT CONSIDERATIONSNON PHARMACOLOGICALOxygen supplementation Pulmonary rehabilitationPHARMACOLOGICALDiscuss therapies with weak no reccomendations with patients based on their individual value