间质性肺炎英文

INTERSTITIAL LUNG DISEASE间质性肺病,Conception of ILD(DPLD),ILD is a spectrum of non-infectious,non-neoplastic diseases involving predominately the alveolar wall and perialveolar tissue and surrounding architecture. DPLD(Diffuse Parenchymal Lung Disease): bronchioles and parenchyma,Medical Terminology,Interstitial lung disease（ILD） 间质性肺病Diffuse parenchymal lung disease(DPLD)弥漫性肺实质肺病Idiopathic interstitial pneumonia（IIP）特发性间质性肺炎Idiopathic pulmonary fibrosis（IPF）特发性肺纤维化Usual interstitial pneumonia(UIP)普通性间质性肺炎Non-specific interstitial pneumonia(NSIP)非特异性间质性肺炎Cryptogenic organizing pneumonia （COP）隐原性机化性肺炎Acute interstitial pneumonia (AIP)急性间质性肺炎Desquamative interstitial pneumonia (DIP)脱屑性间质性肺炎,Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)呼吸细支气管炎间质性肺炎Lymphocytic interstitial pneumonia (LIP)淋巴细胞性间质性肺炎Granulomatous肉芽肿的Sarcoidosis结节病Lymphangioleiomyomatosis淋巴管平滑肌瘤Histiocytosis(HX)组织细胞增多症Reticular网格状Nodule(nodular)结节Ground glass opacity(GGO)磨(毛)玻璃影Bronchiectasis支气管扩张Honeycombing蜂窝Diffusion capacity弥散量Bronchoalveolar lavarge(BAL)肺泡灌洗Transbronchoscopic lung biopsy(TBLB)经气管镜肺活检Crackle爆裂音,Classification of ILD by ATS/ERS 2002,IDIOPATHIC INTERSTITIAL PNEUMONIA(IIP),GRANULOMATOSISsarcoidosisHypersensitive pneumonitisWegenersgranulomatosis,RARE ILDteinosisalv.hemorrhage-nephritis syndromeLangerhans cell histiocytosisLymphangioleiomyomatosis Idiopathic pulmonaryHemosiderosisChronic eosinophilic pneumonia,ILD of known causesOccupational lung disease(pneumoconiosis)Drug-induced lung diseaseConnective disease associated ILD,ILD of Unknown Causes,AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE VOL 165 2002,EPIDEMIOLOGY,ILD accounts for 100,000 hospital admissions yearly15% of patients seen by pulmonologists nationwide. incidence and prevalence of ILD (5/100,000) in the USNew Mexico: overall prevalence of ILD was 80.9 per 100,000 in males and 67.2 per 100,000 in femalesincidence of ILD was 31.5 per 100,000 in men and 26.1 per 100,000 in women. Idiopathic pulmonary fibrosis represented 45% of the patient base.,CLINICAL EVALUATION-History,Length Of Illness And Clinical Course,Acute onset: days to weeksAcute interstitial pneumoniaAcute pneumonitis from collagen vascular disease(especially SLE)Cryptogenic organizing pneumoniaDrugsDiffuse alveolar hemorrhage（弥漫性肺抛出血）Eosinophilic lung disease（嗜酸性粒细胞肺病）Hypersensitivity pneumonitis（过敏性肺炎）Subacute: weeks to monthsCollagen vascular diseaseassociated ILDCryptogenic organizing pneumoniaDrugsSubacute hypersensitivity pneumonitis,Chronic: months to yearsIdiopathic pulmonary fibrosisChronic hypersensitivity pneumonitisCollagen vascular diseaseassociated ILDNonspecific interstitial pneumoniaOccupation-related lung disease (e.g., silicosis,asbestosis),CLINICAL EVALUATION-History,Occupational and Environmental History: hypersensitivity pneumonitis,asbestosisDrug History:amiodaron，bleomycin，radiotherapyAge and Gender: young-sarcoidosis,IPF-50; women-lymphagioleiomyositosis（LM, 淋巴管平滑肌瘤病）; men-pneumoconiosis （尘肺）Smoking History: high-eosinophilic granuloma,RB-ILD, IPF,asbestosis, ；low-hypersensitive pneumonitis(HP过敏性肺炎), sarcoidosisFamily History：familial IPF, Lymphangioleiomyomatosis,Respiratory Symptoms and Signs,Dyspnea:Progressive dyspnea, exertional/resting： the most common complaint. 10% ILD may present with dyspnea with a normal chest radiograph. Suspection after exclusion of COPD,Pulmonary Embolism(肺栓塞).Cough: IPF, sarcoidosis,HP,COPChest Pain: CTD-ILD, pneomothorax with LMWheezing:sarcoidosis,HP,EP,Bibasilar inspiratory crackles(爆裂音): characteristic physical sign in ILD. Mechanism. Dry ralesDigital clubbing,(杵状指) a marker of advanced fibrotic disease, often in IPF; CA suspected if after.,Respiratory Symptoms and Signs,Laboratory Investigation,Autoantibodies (rheumatoid factor, antinuclear factors) CVD, IPF, WG, MPA, NSIPSerum angiotensin converting enzyme(血管紧张素转化酶抑制剂）: Sarcoidosis Eosinophilia : EPAntibasement membrane antibody: GPSAntineutrophilic cytoplasmic antibody(ANCA,抗中性粒细胞胞浆抗体): WG, MPAIncreased serum LDH: IPF, PAP, AIP,Radiographic Features,Grouns-glass opacipation磨玻璃影Reticular or nodular网格/结节影Honey-combing蜂窝影Cyst囊 检查方式Plain CXRPlain CTHRCT：crucial for ILD diagnosis,Alveolar filling pattern肺泡填塞征,Nodular pattern,Honeycomb,Reticular pattern-HP,Nodule-Sarcoidosis,Honey-combing蜂窝肺,IPF,GGO with thickened interlobular septa-crazy paving fashion(铺路石征）,pulmonary alveolar proteinosis,Ground-glass opacity-idiopathic pulmonary hemosiderosis,Cyst囊,Lymphangioleiomyomatosis,Bronchoalveolar Lavage肺泡灌洗,Diagnosis: E35% eosinophilic pneumonia 嗜酸性粒细胞肺炎 periodic acidSchiff (PAS)+: alveolar proteinosis肺泡蛋白沉积正BAL lymphocytes ( 35%) sarcoidosis, HP, drug-induced ILDResponse to therapy: lymphocytosis,BAL-Alveolar proteinosis,Physiologic Testing,Restriction限制性通气功能障碍Diffusion defect弥散量降低Preservation of airflowIncrease in P(A-a)O2Exercise-induced hypoxaemia低氧血症Hyperventilation过度通气,Pulmonary Function Test,Lung Biopsy-definitive diagnosis,The final step in the diagnostic evaluation of a patient with ILD is to decide whether it is necessary to obtain lung tissue.Tranbronchial Lung Biopsy经气管镜肺活检Percutaneous lung biopsy经皮肺活检Video-assited thoracosopic lung biopsy胸腔镜活检Open lung biopsy开胸肺活检,Diagnosis,Multi-disciplinary approach to the diagnosis of ILD with Pulmonologist, radiologist and pathologist.,IDIOPATHIC PULMONARY FIBROSIS(ipf),特发性肺纤维化,IDIOPATHIC INTERSTITIAL PNEUMONIA(IIP)特发性间质性肺炎,The IIPs are a heterogeneous group of nonneoplastic disorders resulting from damage to the lung parenchyma by varying patterns of inflammation and fibrosis.Idiopathic indicates unknown cause and interstitial pneumonia refers to involvement of the lung parenchyma by varying combinations of fibrosis and inflammation, in contrast to airspace disease typically seen in bacterial pneumonia.,IIP的生存曲线,IPF-Definition,IPF is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of UIP defined below . The definition of IPF requires the exclusion of other forms of interstitial pneumonia including other diopathic interstitial pneumonias and ILD associated with environmental exposure, medication, or systemic disease.,Pathology of IPF,Potential Risk Factors,Cigarette smokingEnvironmental factorMicrobial agentsGastroesophageal reflux,Clinical Features,Symptoms&SignsExertional dyspneaCoughBasilar crackle,Lab&other test,DEFINITION OF UIP PATTERN,UIP征象,UIP可能征象,非UIP征象,OTHER TESTS,Pulmonary function test：restriction +diffusion defect with decreased complianceArterial Blood Gas Analysis：Increased P(A-a)，Exertional hypoxaemia with hypocapniaBronchoscopy：tranbronchoscopic lung biopsy（TBLB，经气管镜肺活检）：exclusion of other diseasesLung biopsy：percutaneous，thoracoscopy，open-lungbiopsy,Diagnostic Criteria,The diagnosis of IPF requires the following:1. Exclusion of other known causes of ILD (e.g., domestic and occupational environmental exposures, connective tissue disease, and drug toxicity).2. The presence of a UIP pattern on HRCT in patients not subjected to surgical lung biopsy .3. Specific combinations of HRCT and surgical lung biopsy pattern in patients subjected to surgical lung biopsy .,COMBINATION OF HRCT AND SURGICAL LUNG BIOPSY FOR THE DIAGNOSIS OF IPF,IPF诊断标准,未,IPF诊断标准,未,IPF诊断标准,未,Features associated with increased mortality in IPF,Baseline factorsLevel of dyspneahoneycombing on HRCTPulmonary hypertensionDLCO , 40% predictedDesaturation 10% absolute valueDecrease in DLCO by 15% absolute valueWorsening of fibrosis on HRCT,Acute Exacerbation of IPF（AEIPF）,Criteria for AEIPF have included an unexplained worsening of dyspnea within 1 month, evidence of hypoxemia as defined by worsened or severely impaired gas exchange, new radiographic alveolar infiltrates, and an absence of an alternative explanation such as infection, pulmonary embolism, pneumothorax, or heart failure.,Therapy for IPF,IPF Evidence-Based Treatment:,Strong recommendation against the use:Corticosteroid mono therapy (very low)Colchicine (very low)秋水仙碱Cyclosporine A (very low)环孢霉素Combined corticosteroid and immune-modulator therapy (low)激素+免疫调节剂Interferon gamma 1b (high)干扰素Bosentan (moderate)波生坦,IPF药物治疗,强烈不推荐下列治疗方案:单一激素疗法秋水仙碱环保霉素A激素+免疫抑制剂INF gamma 波生坦,IPF药物治疗,不主张推荐下列药物:乙酰半胱氨酸+激素+免疫抑制剂乙酰半胱氨酸抗凝治疗吡啡尼酮,乙酰半胱氨酸对IPF的作用 IFIGENIA,Demedts et al. NEJM 2005,吡啡尼酮对FVC的影响 CAPACITY 1,P = 0.005*,*Rank ANCOVA (PFD 2403 mg/d vs. placebo at Week 72),P. Noble et al, ATS 2009,吡啡尼酮对FVC的影响 CAPACITY 2剂量反应曲线,Change in % Predicted FVC,P. Noble, ATS 2009,吡啡尼酮对无恶化IPF生存的影响-荟萃分析,吡有效 吡无效,华法令与IPF,INF-与IPF,Interpretation We cannot recommend treatment with interferon gamma-1b since the drug did not improve survival for patients with idiopathic pulmonary fi brosis, which refutes previous fi ndings from subgroup analyses of survival in studies of patients with mild-to-moderate physiological impairment of pulmonary function.,其它治疗,氧疗与对症治疗康复肺移植中医中药,Nature History of IPF,Sarcoidosis,Pulmonary Sarcoidosis,Sarcoidosis is amultisystem disorder of unknownorigin characterized by noncaseating granulomatous inflammation at sites of disease.Lung and intrathoracic lymph nodes most commonly affected,结节病的发展进程,肉芽肿性炎症,吸收,复发,治疗/观察,宿主因素,纤维化并不可逆改变,CLINICAL FEATURES,The hilar glands and the lungs are the organs most commonly affected in sarcoidosis and intrathoracic involvement is the most frequent accompaniment of sarcoidosis affecting other organs.Age and sex: the highest incidence in the third and fourth decades , with a vari