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1、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,I
2、nterstitial 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 （C
3、OP）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
4、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
5、PNEUMONIA(IIP),GRANULOMATOSIS sarcoidosis Hypersensitive pneumonitis Wegeners granulomatosis,RARE ILD alv.proteinosis alv.hemorrhage-nephritis syndrome Langerhans cell histiocytosis Lymphangioleiomyomatosis Idiopathic pulmonary Hemosiderosis Chronic eosinophilic pneumonia,ILD of known causes Occupat
6、ional lung disease(pneumoconiosis) Drug-induced lung disease Connective 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 yearly 15% of patients seen by pulmonologists nationw
7、ide. incidence and prevalence of ILD (5/100,000) in the US New Mexico: overall prevalence of ILD was 80.9 per 100,000 in males and 67.2 per 100,000 in females incidence 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
8、.,CLINICAL EVALUATION-History,Length Of Illness And Clinical Course,Acute onset: days to weeks Acute interstitial pneumonia Acute pneumonitis from collagen vascular disease(especially SLE) Cryptogenic organizing pneumonia Drugs Diffuse alveolar hemorrhage（弥漫性肺抛出血） Eosinophilic lung disease（嗜酸性粒细胞肺病）
9、 Hypersensitivity pneumonitis（过敏性肺炎） Subacute: weeks to months Collagen vascular diseaseassociated ILD Cryptogenic organizing pneumonia Drugs Subacute hypersensitivity pneumonitis,Chronic: months to years Idiopathic pulmonary fibrosis Chronic hypersensitivity pneumonitis Collagen vascular diseaseass
10、ociated ILD Nonspecific interstitial pneumonia Occupation-related lung disease (e.g., silicosis,asbestosis),CLINICAL EVALUATION-History,Occupational and Environmental History: hypersensitivity pneumonitis,asbestosis Drug History:amiodaron，bleomycin，radiotherapy Age and Gender: young-sarcoidosis,IPF-
11、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), sarcoidosis Family History：familial IPF, Lymphangioleiomyomatosis,Respiratory Symptoms and Signs,Dyspnea:Progressive
12、 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,COP Chest Pain: CTD-ILD, pneomothorax with LM Wheezing:sarcoidosis,HP,EP,Bibasilar inspirat
13、ory crackles(爆裂音): characteristic physical sign in ILD. Mechanism. Dry rales Digital 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
14、, MPA, NSIP Serum angiotensin converting enzyme(血管紧张素转化酶抑制剂）: Sarcoidosis Eosinophilia : EP Antibasement membrane antibody: GPS Antineutrophilic cytoplasmic antibody(ANCA,抗中性粒细胞胞浆抗体): WG, MPA Increased serum LDH: IPF, PAP, AIP,Radiographic Features,Grouns-glass opacipation磨玻璃影 Reticular or nodular网格
15、/结节影 Honey-combing蜂窝影 Cyst囊 检查方式 Plain CXR Plain CT HRCT：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,Grou
16、nd-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 ILD Response 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 airflow Increase in P(A-a)O2 Exercis
