复旦大学：《内科学 Internal Medicine MBBS》课程教学资源（课件讲稿）呼吸系统_Interstitial Lung Disease and Sarcoidosis

Interstitial Lung Diseases Yang,Dong(杨冬) yang dong @zs-hospitalshcn Department of Pulmonary medicine, Zhongshan Hospital, Fudan Universtiy

Interstitial Lung Diseases Yang，Dong (杨冬) yang.dong@zs-hospital.sh.cn Department of Pulmonary Medicine, Zhongshan Hospital, Fudan Universtiy

Definition a group of disease which predominantly affect the pulmonary connective tissue and interstitium (between the air sacs of the lungs) of the alveolar walls and share a common response of the lung to injury: alveolitis; inflammation; fibrosis of the interalveolar septum also known as"interstitial pulmonary fibrosis or diffuse parenchymal lung disease dpld)

Definition a group of disease which predominantly affect the pulmonary connective tissue and interstitium (between the air sacs of the lungs) of the alveolar walls and share a common response of the lung to injury: alveolitis; inflammation; fibrosis of the interalveolar septum  also known as―interstitial pulmonary fibrosis‖or ―diffuse parenchymal lung disease (DPLD)‖

Common clinical feature 1. Exertional dyspnea or nonproductive cough 2. Tachypnea and bibasilar end-inspiratory dry cracks 3. Bilateral diffuse infiltrates on chest radiographs 4. Presents as a rEStRICTIVE lung disease DLcol, PA2O2↑ 5. Varying degrees of fibrosis and inflammation, with or without evidence of granulomatous or secondary vascular changes

Common clinical feature 1. Exertional dyspnea or nonproductive cough 2. Tachypnea and bibasilar end-inspiratory dry cracks 3. Bilateral diffuse infiltrates on chest radiographs 4. Presents as a RESTRICTIVE lung disease, DLco↓, PA-aO2↑ 5. Varying degrees of fibrosis and inflammation, with or without evidence of granulomatous or secondary vascular changes

What is the cause of ILD? ◆>180 kinds Chief known cause(1/3) Inhale inorganic dusts Inhale organic dusts P RadIo-active injury Micro-organic infection Drugs > Lymphocytic carcinoma Pulmonary edema

What is the cause of ILD? >180 kinds Chief known cause (1/3) Inhale inorganic dusts Inhale organic dusts Radio-active injury Micro-organic infection Drugs Lymphocytic carcinoma Pulmonary edema

What is the cause of IlD? 今 Chief unknown cause >Primary lung diseases >ILD associated with system disorder(Ctds) >Alveolar filling disorder >ILD associated with pulmonary vasculitis >Inherited disorders

What is the cause of ILD? Chief unknown cause Primary lung diseases ILD associated with system disorder (CTDs) Alveolar filling disorder ILD associated with pulmonary vasculitis Inherited disorders

Classfication-1 L.According to process: Acute/Subacute/Chronic 2. According to cause: Clear /Not clear 3. According to pathology Granulomatous disease /non-tumor and non inflammatory diseases /non-specific diseases occupational diseases /hyperplastic and tumourous diseases /interstitial lung diseases and honey lung

Classfication-1 1.According to process: Acute/Subacute/Chronic 2. According to cause: Clear /Not clear 3. According to pathology Granulomatous disease / non-tumor and non￾inflammatory diseases / non-specific diseases / occupational diseases / hyperplastic and tumourous diseases / interstitial lung diseases and honey lung

Classfication-2 (According to pathology o Interstitial lung Diseases Iusual interstitial pneumonia (UIP 2. nonspecific interstitial pneumonia(NIP) 3. respiratory bronchiolitis (rB) organizing pneumonia(BOOP) 5. diffuse alveolar damage 6. desquamative interstitial pneumonia IP) 7. lymphocytic interstitial pneumonia LIP)

Classfication-2 (According to pathology ) Interstitial lung Diseases 1.usual interstitial pneumonia (UIP) 2.nonspecific interstitial pneumonia (NIP) 3. respiratory bronchiolitis (RB) 4. organizing pneumonia (BOOP) 5. diffuse alveolar damage 6. desquamative interstitial pneumonia (DIP) 7. lymphocytic interstitial pneumonia (LIP)

Pathogenesis . The lung heals in a predictable, consistent manner .g Causes may differ but pathogenesis remains the same Cause= insult, injury, infection, toxin, etc >Cause is what differentiates the individual diseases and their treatments leads to alveolitis-initial event in all interstitial lung diseases

Pathogenesis The lung heals in a predictable, consistent manner Causes may differ but pathogenesis remains the same Cause = insult, injury, infection, toxin, etc Cause is what differentiates the individual diseases and their treatments leads to alveolitis→initial event in all interstitial lung diseases

Injury Alveolitis? the presence of immune cells within the alveolar walls 今 Immune cells > Secrete cvtokines recruiting more immune cells and triggering complex chain of events called“in们 ammation” Cause direct tissue injury Generate oxygen free radicals >Release proteases

Injury Alveolitis?- the presence of immune cells within the alveolar walls Immune cells Secrete cytokines recruiting more immune cells and triggering complex chain of events called ―inflammation‖ Cause direct tissue injury Generate oxygen free radicals Release proteases

Pathogenesis Alveolar wall and tissue damage lead to lea kage of a fibrin-rich exudate into the alveolar spaces

Pathogenesis Alveolar wall and tissue damage lead to leakage of a fibrin-rich exudate into the alveolar spaces