间质性肺炎英文课件.ppt

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 variable female predominance. Not diagnostic Pulmonary: cough, expectoration of sputun, breathlessness, hemoptysis Extrathoracic: skin(erythema nodosum), neurological, cardiac, etc Diagnosis by accident CLINICAL FEATURES Lab test Haemopoietic system: increased ESR, anemia, hypercalcimia, increased LDH, increased activity of serum angiotensin-converting enzyme Tuberculin test: negative Kevin test: positive BAL:lymphocytosis(active 28%), increased ratio of CD4/CD8 Biopsy: lymph node or lung Chest X-ray Hilar adenopathy: the right paratracheal, aortopulmonary window and the hilar. Interstitial lung disease: reticulo-nodular/ground-glass,/alveolar opacity, upper lobe Pleural effusion20% Other tests: Ga67 scan PET Stage I Stage II Stage III Satge IV Diagnosis Radiologgy Biopsy Kvein test PPD Hypercalcimia, hyperuro Active SACE Differential Diagnosis Lymphoma Tuberculosis Lung cancer Other granulomatous disease: pneumoconiosis, hypersensitive pneumonia, etc Indications for Therapy Spontaneous remission 70% I,II Ocular\neurologic\kidney\heart sarcoidosis Symptomic with II stage or higher Progressive decline in lung function Malignant hypercalcimia Prednisone 30-40mg/qd—1-3month 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