Interstitial Lung Disease Flashcards
What is interstitial lung disease
Conditions that affect lung parenchyma diffusely, characterised by progressive interstitial fibrosis + inflammation
Interstitial lung disease clinical features
Dyspnoea on exertion
Non-productive paroxysmal cough
Abnormal breath sounds + CXR/CT
Restrictive spirometry with decreased DLCO
Interstitial lung disease pathology
Fibrosis + remodelling of interstitium
Chronic inflammation
Hyperplasia of type II epithelial cells/pneumocytes
ILD classification
Known cause
Systemic disorders
Idiopathic
What is idiopathic pulmonary fibrosis
Commonest cause of ILD, inflammatory cell infiltrates + fibrosis of unknown cause
Idiopathic pulmonary fibrosis signs + symptoms
Dry cough
Exertional dyspnoea
Malaise, weight loss
Arthralgia
Cyanosis
Clubbing
Fine end-inspiratory crepitations
Idiopathic pulmonary fibrosis complications
Resp failure
Inc lung cancer risk
Idiopathic pulmonary fibrosis tests
Hypoxic, hypercapnic if severe on ABG
Inc CRP, ANA in some
CXR shows dec lung volume, bilateral lower zone reticulo-nodular shadows
CT shows honeycomb lung in advanced disease
Lung biopsy may be needed for diagnosis, usual interstitial pneumonia describes biopsy findings
Idiopathic pulmonary fibrosis management
Supportive care
Trials + transplant
In future:
Nintedanib (targets growth factors)
Pirfenidone (immunosuppressant and anti-fibrotic)
Idiopathic pulmonary fibrosis prognosis
50% 5yr survival