文档介绍：Interstitial lung disease
ZHAO LI (赵 立)
Pulmonary interstitium
Definition
The supporting structure between alveolus or extra-epithelium of terminal bronchiole, involving blood vessels and lymphatic tissue.
Components
Cells
Inflammatory and immunologic cells:
Mononuclear macrophage (~90%), lymphocyte (~10%)
Extracellular matrix (ECM)
Interstitial lung disease
A group of diseases due to damage and repair of pulmonary intrstitium, alveoli and /or bronchiole.
Clinical findings:
Gradually aggravated exertional dyspnea
Restrictive ventilatory disorder
Lower diffusion capacity
Hypoxemia
Diffusion lesion in chest radiograph
Etiology
Known causes
Unknown causes
Inorganic dust (asbestos, etc. 石棉 ）
Organic dust （mushroom dust, pigeon droppings, etc.)
Drugs (Bleomycin, etc.)
Therapy (radiation, oxygen toxicity)
Infection (virus, mycoplasma, etc.)
Non-pulmonary diseases (pulmonary edema, renal failure)
Idiopathic interstitial pneumonia (idiopathic pulmonary fibrosis, etc.)
Sarcoidosis
Connective tissue disease related ILD
Pulmonary vasculitis related ILD (Wegner’s granuloma, etc.)
pulmonary alveolar proteinosis
Pathogenesis
Fibrogenic Stimulus
Macrophage & Monocyte Activation
Cytokines
T Cell Activation
Cytokines
Fibroblasts
Cell Proliferation & Extracellular Matrix Synthesis
FIBROSIS
Diagnosis
History (occupation, environment, etc.)
Underlying diseases
Chest radiograph (high resolution CT, HRCT)
Pulmonary function tests (restrictive, lower DLCO, hypoxemia)
Bronchoalveolar lavage fluid (BALF)
Biopsy (open lung biopsy, transbronchial lung biopsy, TBLB)
Systemic physical examination (CTD, Wegner’s)
Idiopathic pulmonary fibrosis
Definition
Idiopathic interstitial pneumonia
The pathologic diagnosis of idiopathic pulmonary fibrosis (IPF) is UIP.
The incidence of IPF in IIP is about 47~71%.
usual interstitial pneumonitis (UIP)
nonspecific interstitial pneumonitis (NSIP)
bronchi