Respiratory: Interstitial Lung Disease Flashcards
What is the pathophysiology of ILD?
Repeated cycles of ep inflammation by some unidentified agent = dysregulated repair process Inflam pathway also promotes fibrosis
Outline the aetiology of ILD
Occupational = asbestosis, silicosis, coal workers pneumoconiosis, hypersensitivity pneumonitis (birds)
RA, SLE, scleroderma, radiation therapy
Medication = amiodarone, methotrexate, nitrofurantoin, bleomycin, radiation, cyclophosphamide
Environmental = inorganic or organic dusts, radiation
What are the signs and symptoms of ILD?
Progressive breathlessness
Dry cough
Finger clubbing
Cor-pulmonale
Fine inspiratory crackles
Cyanosis
What investigations should be performed for suspected ILD?
CXR - reticular (thready lines) nodular pattern
HRCT = reticulonodular shadowing, ground glass appearance, honeycombing (end-stage)
Pulse oximetry - sats
ABGs (stats <92)
Lung biopsy (young, atypical presentation, atypical radiology, rule out malignancy)
Six min walk test
ECHO
Immunological screen
Avian precipitins
Serum ACE levels
Pulmonary function test =
o TLC = lowered due to fibrosis
o FVC = low
o FEV1 = normal as problem is not in the airway - increased FEV1/FVC ratio
o DLCO = low due to fibrosis and transfer being impaired
How is ILD managed?
Antifibrotic agents - perfenidone and nintedanib = for pts with FVC between 50-80%
Oxygen
Abx
Diuretics
Pulmonary rehabilitation
Lung transplant for selected younger pts
What are the complications of ILD?
Median survival = 3-5yrs 75% die from resp failure
1/10 will develop lung cancer
What are the common types of ILD?
Idiopathic pulmonary fibrosis.
Non - specific interstitial pneumonia.
Desquamative interstitial pneumonia.
Respiratory bronchiolitis/interstitial lung disease.
Acute interstitial pneumonia.
Lymphoid interstitial pneumonia.
Cryptogenic organising pneumonia.
How does ILD appear on CXR?
How does ILD appear on CT?
