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1、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 parenchymaMedical TerminologyInterstitial lung disease（ILD）间质性肺病Dif
2、fuse parenchymal lung disease(DPLD)弥漫性肺实质肺病Idiopathic interstitial pneumonia（IIP）特特发性间质性肺炎发性间质性肺炎Idiopathic pulmonary fibrosis（IPF）特发特发性肺纤维化性肺纤维化Usual interstitial pneumonia(UIP)普通性间普通性间质性肺炎质性肺炎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 inter
3、stitial 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结节病结节病Lymphangioleiomyomatosis淋巴管平滑肌瘤Histiocytosis(HX)组织细胞增多症Ret
4、icular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 2002IDIOPATHIC INTERSTITIAL PNEUMONIA(IIP)GRANULOMATOSISsarcoidosisHy
5、persensitive pneumonitisWegenersgranulomatosis,RARE ILDalv.proteinosisalv.hemorrhage-nephritis syndromeLangerhans cell histiocytosisLymphangioleiomyomatosis Idiopathic pulmonaryHemosiderosisChronic eosinophilic pneumoniaILD of known causesOccupational lung disease(pneumoconiosis)Drug-induced lung di
6、seaseConnective disease associated ILDILD of Unknown CausesAMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE VOL 165 2002EPIDEMIOLOGYILD accounts for 100,000 hospital admissions yearly15%of patients seen by pulmonologists nationwide.incidence and prevalence of ILD(5/100,000)in the USNew Mex
7、ico: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-HistoryLength Of Illness And Clinical CourseAcute
8、 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 dis
9、easeassociated ILDCryptogenic organizing pneumoniaDrugsSubacute hypersensitivity pneumonitisChronic:months to yearsIdiopathic pulmonary fibrosisChronic hypersensitivity pneumonitisCollagen vascular diseaseassociated ILDNonspecific interstitial pneumoniaOccupation-related lung disease(e.g.,silicosis,
10、asbestosis)CLINICAL EVALUATION-HistoryOccupational 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
11、granuloma,RB-ILD,IPF,asbestosis,；low-hypersensitive pneumonitis(HP过敏性肺炎),sarcoidosisFamily History：familial IPF,LymphangioleiomyomatosisRespiratory Symptoms and SignsDyspnea:Progressive dyspnea,exertional/resting：the most common complaint.10%ILD may present with dyspnea with a normal chest radiograp
12、h.Suspection after exclusion of COPD,Pulmonary Embolism(肺栓塞).Cough:IPF,sarcoidosis,HP,COPChest Pain:CTD-ILD,pneomothorax with LMWheezing:sarcoidosis,HP,EPBibasilar inspiratory crackles(爆裂音爆裂音):characteristic physical sign in ILD.Mechanism.Dry ralesDigital clubbing,(杵状指)a marker of advanced fibrotic
13、disease,often in IPF;CA suspected if after.Respiratory Symptoms and SignsLaboratory InvestigationAutoantibodies(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 cyto
14、plasmic antibody(ANCA,抗中性粒细胞胞浆抗体):WG,MPAIncreased serum LDH:IPF,PAP,AIPRadiographic FeaturesGrouns-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 patternHoneycombReticular pattern-HPNodule-
15、SarcoidosisHoney-combing蜂窝肺IPFGGO with thickened interlobular septa-crazy paving fashion(铺路石征）pulmonary alveolar proteinosisGround-glass opacity-idiopathic pulmonary hemosiderosisCyst囊LymphangioleiomyomatosisBronchoalveolar Lavage肺泡灌洗肺泡灌洗Diagnosis:E35%eosinophilic pneumonia 嗜酸性粒细胞肺炎 periodic acidSch
16、iff(PAS)+:alveolar proteinosis肺泡蛋白沉积正BAL lymphocytes(35%)sarcoidosis,HP,drug-induced ILDResponse to therapy:lymphocytosisBAL-Alveolar proteinosisPhysiologic TestingRestriction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P
17、ulmonary Function TestLung Biopsy-definitive diagnosisThe 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
18、胸肺活检DiagnosisMulti-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 paren
19、chyma 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-Def
20、initionIPF 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 exclusi
21、on of other forms of interstitial pneumonia including other diopathic interstitial pneumonias and ILD associated with environmental exposure,medication,or systemic disease.Pathology of IPFPotential Risk FactorsCigarette smokingEnvironmental factorMicrobial agentsGastroesophageal refluxClinical Featu
22、resSymptoms&SignsExertional dyspneaCoughBasilar crackle Lab&other testDEFINITION OF UIP PATTERNUIP征象UIP可能征象非UIP征象OTHER TESTSPulmonary function test：restriction+diffusion defect with decreased complianceArterial Blood Gas Analysis：Increased P(A-a)，Exertional hypoxaemia with hypocapniaBronchoscopy：tra
23、nbronchoscopic lung biopsy（TBLB，经气管镜肺活检）：exclusion of other diseasesLung biopsy：percutaneous，thoracoscopy，open-lungbiopsyDiagnostic CriteriaThe diagnosis of IPF requires the following:1.Exclusion of other known causes of ILD(e.g.,domestic and occupational environmental exposures,connective tissue di
24、sease,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
25、断标准未未IPF诊断标准未未IPF诊断标准未未Features associated with increased mortality in IPFBaseline factorsLevel of dyspneahoneycombing on HRCTPulmonary hypertensionDLCO,40%predictedDesaturation 10%absolute valueDecrease in DLCO by 15%absolute valueWorsening of fibrosis on HRCTAcute Exacerbation of IPF（AEIPF）Criteri
26、a 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 fa
27、ilure.Therapy for IPFIPF Evidence-Based Treatment:Strong recommendation against the use:Corticosteroid mono therapy(very low)Colchicine(very low)秋水仙碱秋水仙碱Cyclosporine A(very low)环孢霉素环孢霉素Combined corticosteroid and immune-modulator therapy(low)激素激素+免疫调节剂免疫调节剂Interferon gamma 1b(high)干扰素干扰素Bosentan(mod
28、erate)波生坦波生坦IPF药物治疗强烈不推荐下列治疗方案强烈不推荐下列治疗方案:单一激素疗法单一激素疗法秋水仙碱秋水仙碱环保霉素环保霉素A激素激素+免疫抑制剂免疫抑制剂INF gamma 波生坦波生坦IPF药物治疗不主张推荐下列药物不主张推荐下列药物:乙酰半胱氨酸乙酰半胱氨酸+激素激素+免疫抑制剂免疫抑制剂乙酰半胱氨酸乙酰半胱氨酸抗凝治疗抗凝治疗吡啡尼酮乙酰半胱氨酸对乙酰半胱氨酸对IPFIPF的作用的作用 IFIGENIA IFIGENIADemedts et al.NEJM 2005Demedts et al.NEJM 2005吡啡尼酮对对FVCFVC的影响的影响 CAPACITY 1C
29、APACITY 1P=0.005*Rank ANCOVA(PFD 2403 mg/d vs.placebo at Week 72)P.Noble et al,ATS 2009吡啡尼酮对对FVC的影响的影响 CAPACITY 2剂量反应曲线剂量反应曲线Change in%Predicted FVCP.Noble,ATS 2009 吡啡尼酮对无恶化IPF生存的影响-荟萃分析吡吡有效有效 吡吡无效无效 华法令与IPFINF-与IPFInterpretation We cannot recommend treatment with interferon gamma-1b since the drug
30、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 IPFSarcoidosisPulmonary Sarc
31、oidosisSarcoidosis 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 FEATURESThe hilar glands and the lungs are the organs mos
32、t 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 variable female predominance.Not diagnosticPulmonary:cough,expectoration of sputun,breath
33、lessness,hemoptysisExtrathoracic:skin(erythema nodosum),neurological,cardiac,etcDiagnosis by accidentCLINICAL FEATURESLab testHaemopoietic system:increased ESR,anemia,hypercalcimia,increased LDH,increased activity of serum angiotensin-converting enzymeTuberculin test:negative Kevin test:positiveBAL:
34、lymphocytosis(active 28%),increased ratio of CD4/CD8Biopsy:lymph node or lung Chest X-rayHilar adenopathy:the right paratracheal,aortopulmonary window and the hilar.Interstitial lung disease:reticulo-nodular/ground-glass,/alveolar opacity,upper lobePleural effusion70%I,IIOcularneurologickidneyheart
35、sarcoidosisSymptomic with II stage or higherProgressive decline in lung functionMalignant hypercalcimiaPrednisone 30-40mg/qd1-3month tapering to 15mg for 6-9month,tapering with 2.5mg/wk maintainance for 1-2year结节病疗程Judson MA,The treatment of pulmonary sarcoidosis,Respiratory Medicine(2012),doi:10.1016/j.rmed.2012.01.013结节病激素疗程治疗示意图