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Interstitial Lung Disease
Asma Albtoosh ,MD
Respiratory and sleep medicine
JUH
What is interstitial lung disease ?
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Pathogenesis of ILDs
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Mosby items and derived items © 2009 by Mosby,
Inc., an affiliate of Elsevier Inc. 4
Pathophysiology
• Repeated exposure to inflammatory agents or imperfect repair of damaged tissue leads to permanent damage.
• Physiological impairment due to damage• V/Q mismatch, shunt, ↓DLCO
• Decreased lung compliance .
. .
Definition
Group of pulmonary disorders characterized clinically by respiratory symptoms associated with :
• Radiologically diffused infiltrates.• Histologically by distortion of the gas exchanging units.• Physiologically by restriction of lung volumes and impaired
oxygenation.
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Secondary pulmonary lobule
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ClassificationATS/ERS
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Mosby items and derived items © 2009 by Mosby,
Inc., an affiliate of Elsevier Inc. 8
Diagnosis
History
• Age >70 years ……… IPF(Idiopathic pulmonary fibrosis ).
• Exertional dyspnea and nonproductive cough
• Rarely sputum production, hemoptysis, or wheezing
Duration :
Acute : AIP
Infection
Acute HP(hypersensitivity pneumonitis ), acute EP (eosinophilic pneumonia).
Drug reaction COP, CTD (e.g. acute lupus pneumonitis)
DAH (diffuse alveolar hemorrhage ).
Chronic :IPF ,
• Non respiratory symptoms related to disease complications .
• Smoking related ILDs( Interstitial lung diseases).
• Exposure and occupation .
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Hypersensitivity pneumonitis (HP)
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Physical examination
Chest Assessment FindingsGeneral : Cyanosis ,etc…will show you pictures .
Chest : Increased tactile and vocal fremitus
Dull percussion note
Bronchial breath sounds
Crackles, usually fine end inspiratory
Pleural friction rub
Whispered pectoriloquy.
Chest ultrasound US ?
B lines
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Velcro crackles Fine end inspiratory
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Hermansky pudlak syndrome
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Connective tissue related ILD
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Lab test
• RF(rheumatoid factor)
• ANA(anti nuclear antibody ).
• Anti DS –DNA( Double stranded DNA )
• ENA (Extranuclear antibody ).
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Sarcoidosis
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• Serum ACEI level
• Hypercalcemia
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Dermatomyositis/Polymyositis
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• ANA( antinuclear antibody )
• ENA( Extra nuclear antibody )
• Myositis panel
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IPF(Idiopathic pulmonary fibrosis )Histologically : UIP(usual interstitial pneumonia )Most common form of ILD .
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Granulomatosis with polyangiitis /previously Wegeners
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• ANCA( anti neutrophilic cytoplasmic antibodies )
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Lymphangiolyomyomatosis(LAM)
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Lymphangitis carcinomatosis
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Sarcoidosis
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Asbestosis
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Hypersensitivity pneumonitis
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Congestive heart failure
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Honeycombing IPF
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NSIP(nonspecific interstitial pneumonia)GGO
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Smoking related interstitial lung disease
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Diagnosis ?
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Drug induced Radiation induced
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Lung volumesReduced TLC(total lung capacity )
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Spirometry Low FVCNormal FEV1/FVC ratio
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Multidisciplinary meeting
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Management plan
• A treatment plan should not only consist of pharmacologic agents that are prescribed to prevent progression and/or induce remission
• supportive therapies :
Supplemental oxygen if indicated.
Pulmonary rehabilitation.
Measures to relieve symptoms (e.g. cough)
Treatment of co-morbid conditions (e.g. anemia, sleep disordered breathing, GER, pulmonary hypertension ,infectious complications).
Vaccinations
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Pharmacotherapy
• Systemic steroids : cryptogenic organizing pneumonia (COP), eosinophilic pneumonia, sarcoidosis, or cellular non-specific interstitial pneumonia (NSIP)
• Immunosuppressives: some forms of CTD-associated ILD (NSIP or UIP pathologies) have been reported to respond to mycophenolatetherapy, which also allowed a significant lowering of corticosteroid dosing .
• Antifibrotics: pirfenidone
nintedanib
• Antireflux medications
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Lung transplant
• Lung transplantation is the only form of therapy that may improve quality of life and survival for patients with IPF.
• 5-year survival following lung transplantation for IPF or other forms of pulmonary fibrosis is approximately 50%.
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Thank you
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