2. Bronchiolitis Obliterans with Organizing Pneu- monia (BOOP). HRCT shows multi- focal areas of hazy increase in lung density, and associated peripheral thickening of interlobular septa

3. Alveolar Proteinosis (Alveolar filling disease) Confluent bilateral infiltrates with a mixed institial and ground- glass appearance. Post Rt. Lung lavage 20 L of progressively clearing lavage fluid were drained from the right lung. The initial effluent was thick with chalky white sediment that cleared with successive lavages

4. Diagnosis of IPF Major Criteria Exclusion of other known causes of interstitial lung diseases Abnormal pulmonary function studies : FVC ,FEV1/FVC , AaPO2 , and DLco  HRCT: bibasilar reticular abnormalities with minimal ground-glass opacities TBLB and BAL show no features to support another diagnosis

5. Diagnosis of IPF Minor Criteria: Age >50 yrs Insidious onset of otherwise unexplained exertional dyspnea Duration of illness  3 months Bibasilar inspiratory crackles

6. Current Management of IPF (1) Antiinflammatory therapy: chronic persistent inflammation  fibrosis Antifibrotic therapy: colchicine, D-penicilamine, interferon gamma ( IFN-  ), IFN-  1b, and pirfenidone  decreasing the excessive matrix ( collagen)

7. Current Treatment Recommendation for IPF (2) Corticosteroid ( prednisone or equivalent) 0.5 mg/kg daily for 4wks  0.25 mg/kg /day for 8wks Taper to 0.125mg/kg/day or 0.25mg/kg or alternate days Plus Azathioprine: 2-3mg/kg/day or Cyclophosphamide: 2mg/kg/day. Maximum dose 150mg daily. Dosing should begin at 25-50 mg/day, increasing by 25mg increments every 1-2weeks until the maximum dose is achieved Therapy should be continued for a minimum of 6 months. Response is determined by symptoms, radiologic and physiologic findings.

8. Potential Future Approach to Treatment of IPF At present, the most promising approaches :antioxidants, interferon gamma, and blockade of tumor necrosis factor alpha and transforming growth factor beta. Future possibilities : blockade of cell signaling transduction element and, ultimately, gene transfer blocking strategies

9. Assessing Response to Therapy Clinical improved Two or more of the following on two consecutive visits over a 3 to 6 months period Symptoms: decreased dyspnea and cough Radiology: reduced parenchymal abnormality Physiology: improvement defined by two or more of the following:  10% increase TLC or FVC ( Minimum 200mL),  10% increase in DLco ( minimum 3mL/min/mmHg), significant improvement (  4% point,  4mmHg) or normalization of O2 saturation or PaO2 during formal exercise testing

10. Diagnosis of ILD History: genetic factor, detail work and environmental history Physical findings Laboratory and diagnostic test Pulmonary function test Bronchoscopy Lung biopsy

11. Staging of Disease Activity

12. SLE with lung involvement 50 % develop ultimately Pleuritis, pleural effusion, acute penumonitisfrom pulmonary capillaritis causing alveolar hemorrhage are the most frequent forms of lung disease, while a chronic, progressive ILD is uncommon. Lymphocytic alveolitis may occur: better response to immunosuppressive therapy

13. Rheumatoid Arthritis with Lung Involvement Pleural effusion, subpleural nodules, parenchymal nodular infiltration associated with pneumoconiosis ( Caplan’s syndrome), and diffuse interstitial fibrosis. ILD can develop before joint disease particular in man

14. RA with ILD (Above) Reticulonodular pattern (Lt.)

15. Ankylosing Spondylitis Bil. Upper lobe fibrosis, which can be complicated by fibrocavitary disease, may develop late in the course

16. Systemic Sclerosis with Lung Involvement Involve the anterior chest wall and abdomen: restrictive lung function Distal esophageal motor dysfunction: Regurgitation and chronic aspiration is common

17. Sjogren’s Syndrome with Lung Involvement General dryness and lack of airways secretions cause the major problems of hoarseness, cough, and bronchitis. Lyphocytic infiltrate: ILD ( low grade lymphoma) Bronchiolitis obliterans: affect small terminal airways  lung hyperinflation

18. Polymyositis and Dermatomyositis with Lung Involvement 5-10 % polymyositis and dermatomyositis. Weakness of respiratory muscles  aspiration pneumonitis

19. Syndrome of ILD with Pul. Hemorrhage Recurrent hemoptysis, dyspnea, and hypoxemia  diffuse alveolar opacities  suspect alveolar hemorrhage Etiology: SLE; wegener’s granulomatosi ; behcet’s disease; allergic Chur-Strauss granulomatosis; Henoch-Schonlein purpura syndrome; Essential ( mixed) cryoimmunoglobulinemia.

20. 謝謝聽講 敬請指教 Thank You for Your Attention