Bronchiolitis Obliterans with Organizing Pneu- monia (BOOP). HRCT shows multi- focal areas of hazy...
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Transcript of Bronchiolitis Obliterans with Organizing Pneu- monia (BOOP). HRCT shows multi- focal areas of hazy...
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BronchiolitisObliterans withOrganizing Pneu-monia (BOOP). HRCT shows multi-
focal areas of hazyincrease in lung
density, and associatedperipheral thickeningof interlobular septa
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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
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Diagnosis of IPF
• Major Criteria• Exclusion of other known causes of interstitial lung diseas
es• Abnormal pulmonary function studies : FVC,FEV1/FVC
, AaPO2 , and DLco • HRCT: bibasilar reticular abnormalities with minimal gro
und-glass opacities• TBLB and BAL show no features to support another diagn
osis
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Diagnosis of IPF
• Minor Criteria:
• Age >50 yrs
• Insidious onset of otherwise unexplained exertional dyspnea
• Duration of illness 3 months
• Bibasilar inspiratory crackles
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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)
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Current Treatment Recommendation for IPF (2)
• Corticosteroid ( prednisone or equivalent)
0.5 mg/kg daily for 4wks0.25 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.
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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
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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 PaO
2 during formal exercise testing
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Diagnosis of ILD
• History: genetic factor, detail work and environmental history
• Physical findings
• Laboratory and diagnostic test
• Pulmonary function test
• Bronchoscopy
• Lung biopsy
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Staging of Disease Activity
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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
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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
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RA with ILD (Above)
Reticulonodular pattern (Lt.)
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Ankylosing Spondylitis
Bil. Upper lobe fibrosis, which can be complicated by fibrocavitary disease, may develop late in the course
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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
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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
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Polymyositis and Dermatomyositis with Lung Involvement
• 5-10 % polymyositis and dermatomyositis.
• Weakness of respiratory muscles aspiration pneumonitis
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Syndrome of ILD with Pul. Hemorrhage
• Recurrent hemoptysis, dyspnea, and hypoxemia diffuse alveolar opacities suspect alveolar hemo
rrhage
• Etiology: SLE; wegener’s granulomatosi ; behcet’s disease; allergic Chur-Strauss granulomatosis; Henoch-Schonlein purpura syndrome; Essential ( mixed) cryoimmunoglobulinemia.
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謝謝聽講 敬請指教
Thank You for Your Attention