Interstitial and fibrotic lung disease Flashcards
What is interstitial lung disease?
Umbrella term used to describe for a group of diseases which cause fibrosis of the lungs. Most common forms are idiopathic pulmonary fibrosis and hypersensitivity pneumonitis
What are some causes of interstitial lung disease?
By aetiology: lung damage (infarction, pneumonia, TB), irritants, connective tissue disease, medications (amiodarone, nitrofurantoin and bleomycin), IPF, hypersensitivity pneumonitis
Upper locations - HART: hypersensitivity pneumonitis, ankylosing spondylitis, radiotheraptyand TB
Lower location - RAIDS: Rheumatoid arthritis, Asbestosis, Idiopathic, Drugs, Sarcoid
What are the signs and symptoms of interstital lung disease?
Symptoms: dry cough, SOB, fatigue which come on insidious and progressive onset. Must ask about pets, occupation and PMH.
Signs: Cyanosis, clubbing, cachexia, fine inspiratory crackles
What are the investigations for ILD?
Bloods: FBC, U&E, CRP, bone profile, rheumatoid factor, autoimmune screen, scl-70 antibodies, allergens, serum ACE
Chest X ray: reticular and nodular shadowing, bilateral hilar lymphadenopathy in sarcoid.
Spirometry: FEV1/FVC ratio >0.7
High resolution CT: Gold standard for IDL. Findings are honeycombing, ground-glass change and traction bronchiectasis.
Bronchoscopy: Bronchoalveolar lavage to identify predominant cells
Biopsy
What are features of Idiopathic pulmonary fibrosis
Progressive exertional dyspnoea and dry cough that comes on over the course of 3 months.
Bibasal fine end-inspiratory creps,
Clubbing.
What is the diagnosis of idiopathic pulmonary fibrosis?
Spirometry: restrictive pattern.
Impaired gas exchange: reduced transfer factor.
Imaging - first line is high resolution CT. Imaging shows bilateral interstitial shadowing with ground-glass appearance progressing to honeycombing
What is idiopathic pulmonary fibrosis?
Progressive, fibrotic lung disease of unknown cause. There are both genetic and environmental causes
What is the management of idiopathic pulmonary fibrosis?
- Pulmonary rehabilitation,
- Antifibrotic drugs: Pirfenidone, nintedanib.
However despite treatments the median survival is only 3 years so need palliative care
What is hypersensitivity pneumonitis?
Hypersensitivity induced lung damage caused by a variety of inhaled organic particles. It is a type III hypersensitivity reaction.
What are some examples of hypersensitivity pneumonitis?
Bird fanciers’ lung (avian proteins from bird droppings)
Farmers lung
Malt workers’ lung: Aspergillus clavatus
Mushroom workers lung
What is the presentation of of hypersensitivity pneumonitis?
Acute (4-8h) - dyspnoea, dry cough and fever.
Chronic (weeks to months) - Lethargy, dyspnoea, productive cough, anorexia and weight loss
Fine inspiratory crackles
What are the investigations for hyersensitivity pneumonitis?
Imaging - upper/mid zone fibrosis,
Bronchoalveolar lavage - lymphocytosis
Serology for specific IgG antibodies
Bloods - no eosinophilia
What is the management of hypersensitivity pneumonitis?
Avoid precipitating factors and give oral glucocorticoids
What is sarcoidosis?
Multisystem disease characterized by noncaseating granulomas in different organs which imapirs their function. It predominantly affects the lungs and lymph glands
What is the presentation of sarcoidosis?
Acute - erythema nodosum, bilateral hilar lymphadenopathy, swinging fever and polyarthralgia.
Constitutional - fever, weight loss, arthralgia, fever, lymphadenopathy, enlarged parotid glands.
Pulmonary (90%)- Dry cough, dyspnoea, pulmonary fibrosis, crepitation
Neurological - meningitis, peripheral neuropathy, bilateral bell’s palsy.
Ocular - uveitis, keratoconjuctivitis sicca
Cardiac - restrictive cardiomyopathy
Abdominal - hepatosplenomegaly and renal stones, cholestasis.
Derm - erythema nodosum and lupus pernio.
Endo - Hypercalaemia
