9. Interstitial Lung Disease Flashcards
What is interstitial lung disease?
A group of diseases the result in disruption of the acinus due to scarring
Alveoli are gradually replaced by fibrotic tissue, and lose the ability to transfer oxygen into the blood
What are the causes of interstitial lung disease?
Idiopathic Associated with collagen vascular diseases Drug/treatment related Occupational/environmental exposure Post infection
What are the causes of interstitial lung disease in the upper zones?
CHHARTS Coalworkers Hypersensitivity Histiocytosis ankylating spondylitis Radiation TB Silicosis
What are the causes of interstitial lung disease in the lower zones?
RAIDS Rheumatoid Arthritis Asbestosis IPF Drugs Scleroderma
What general ILD symptoms can be picked up when taking a history?
Gradual dyspnoea for 6-12 months
Dry cough
What age group and gender has the highest rate of IPF?
men >60
What age group and gender has the highest rate of sarcoid and connective tissue disease related ILD?
women 20-40
What type of ILD goes away when the patient stops smoking?
Respiratory bronchiolitis
What signs can be seen in the hands in ILD?
Clubbing (IPF, asbestosis) Sclerodactaly (scleroderma) Splinter haemorrhages (RA, SLE) Raynaud's (scleroderma, SLE) Gottron papules (dermatomyositis)
What signs can be seen on the head and neck in ILD?
Butterfly rash (SLE)
Parotid swelling, facial palsy and iritis (sarcoid)
Telangiectasia (scleroderma)
Central cyanosis
What signs can be found in the chest in ILD?
Rapid shallow breathing
Tracheal deviation
Decreased chest diameter and expansion
Tactile fremitus normal or increased
Percussion is normal or dull
Bilateral fine end inspiratory creps ('velcro crackles')What other signs might be seen in ILD?
Erythema nodosum on legs (sarcoidosis)
Myopathy and small joint arthritis (mixed connective tissue disease)
Marks of radiation therapy
What investigations should be done if ILD is suspected?
High res CT thorax CXR PFTs Bloods Functional assessment Bronchoscopy and lung biopsy
What can be seen on a HRCT in ILD?
Coarse reticular opacities
‘Ground glass’ infiltrates
Honeycomb cysts
Traction bronchiectasis (airways are pulled open)
How are infiltrates on a CT differentiated from fluid?
CT is taken with the patient in different positions: if the opacity moves then it is fluid
How is ILD diagnosed from a CXR?
Patchy or nodular infiltrates that start peripherally
No other signs of congestive cardiac failure
What will PFTs show in ILD?
Restrictive pattern on spirometry (decreased FEV1 and FVC)
Reduced diffusion lung capacity
Reduced total lung capacity
What are the causes of an obstructive defect in ILD?
Have both COPD and ILD
Sarcoidosis
What bloods can be done in ILD?
FBC, U&E, liver and bone profiles
Connective tissue disease antibodies
What are the functional assessments used in ILD?
ABG
6 minute walk test
What drugs are given as anti-fibrotics in IPF?
Pirfenidone
Nintedanib
What management is given for patients with IPF?
Oxygen therapy
Lung rehab
Lung transplant if under 65
What can commonly be picked up on CXR in sarcoidosis?
Bihilar lymphadenopathy
What method is used to sample hilar lymph nodes in sarcoidosis?
EBUS-TBNA
What is the combination of bihilar lymphadenopathy and erythema nodosum known as?
Lofgren’s syndrome
What can be found in the blood in sarcoidosis?
Increased Ca and ACE
What other symptoms can be found in sarcoidosis?
Uveitis
Keratoconjunctivits
Rash
hepatic granulomas
What type of drug does sarcoid respond well to?
Steroids eg. prednisone
What type of hypersensitivity is referred to in hypersensitivity ILD?
Type 3: 6-8 hours after exposure
Progresses to type 4 which causes pulmonary fibrosis
What can be seen in the blood in hypersensitivity ILD?
Serum precipitins to antigen
IgE
What are the symptoms of hypersensitivity ILD?
Dry cough, fever, chills, basal creps
What are the pulmonary manifestations of RA?
Fibrotic lung disease
Pulmonary nodules
Pleuritis
Pleural effusion
Is the pleural effusion in RA exudative or transudative?
Exudative
How long after treatment does radiation ILD present?
6 months
What drugs can cause ILD?
Bleomycin, busulphan Methotrexate Nitrofuratoin Amiodarone Prolonged high dose O2
