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 .

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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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