BronchiolitisObliterans withOrganizing Pneu-monia (BOOP). HRCT shows multi-

focal areas of hazyincrease in lung

density, and associatedperipheral thickeningof interlobular septa

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

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

Diagnosis of IPF

• Minor Criteria:

• Age >50 yrs

• Insidious onset of otherwise unexplained exertional dyspnea

• Duration of illness 3 months

• Bibasilar inspiratory crackles

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)

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.

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

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

Diagnosis of ILD

• History: genetic factor, detail work and environmental history

• Physical findings

• Laboratory and diagnostic test

• Pulmonary function test

• Bronchoscopy

• Lung biopsy

Staging of Disease Activity

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

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

RA with ILD (Above)

Reticulonodular pattern (Lt.)

Ankylosing Spondylitis

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

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

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

Polymyositis and Dermatomyositis with Lung Involvement

• 5-10 % polymyositis and dermatomyositis.

• Weakness of respiratory muscles aspiration pneumonitis

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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