Interstitial Lung Disease Flashcards
What is interstitial lung disease?
Group of conditions that cause inflammation +/or fibrosis of the lung parenchyma
What is fibrosis?
Replacement of elastic, functional lung tissue with non-functional scar tissue
Give 5 examples of interstitial lung disease
Idiopathic pulmonary fibrosis
Secondary pulmonary fibrosis
Hypersensitivity pneumonitis
Cryptogenic organising pneumonia
Pneumoconiosis (inc. Asbestosis)
Give 3 symptoms of interstitial lung disease
SOBOE
Dry cough
Fatigue
Describe idiopathic pulmonary fibrosis. What is the life expectancy?
Progressive pulmonary fibrosis with no apparent cause
Life expectancy: 2-5y
Give 2 signs of IPF
Bibasal fine end-inspiratory crepitations
Clubbing
Describe management of IPF
C: Pulmonary rehabilitation + stop smoking
M: Pirfenidone (reduces fibrosis + inflammation) + LTOT
S: lung transplant
Name 4 drugs that can cause secondary pulmonary fibrosis
Amiodarone (+ grey/ blue skin)
Cyclophosphamide
Methotrexate
Nitrofurantoin
Name 5 conditions that can cause secondary pulmonary fibrosis
Alpha-1 antitrypsin deficiency
RhA
SLE
Systemic sclerosis
Sarcoidosis
What is hypersensitivity pneumonitis also known as?
Extrinsic allergic alveolitis
What is hypersensitivity pneumonitis?
Inflammation of alveoli + distal bronchioles caused by an immune response to inhaled allergens
What immune response occurs in hypersensitivity pneumonitis?
Type III + iV hypersensitivity
Give 4 examples of hypersensitivity pneumonitis
Bird fanciers’ lung
Farmers lung
Malt workers’ lung
Mushroom workers’ lung
List 5 symptoms in acute hypersensitivity pneumonitis
Cough
Dyspnoea
Fever
Malaise
Chest tightness
List 4 ways in which chronic hypersensitivity pneumonitis manifests
Insidious cough/ dyspnoea
Weight loss
Clubbing
Widespread fibrotic changes mimicking IPF
What will be heard on auscultation in hypersensitivity pneumonitis?
Bilateral mid-zone inspiratory crepitations
What investigations are performed in suspected hypersensitivity pneumonitis ?
FBC: r/o infection
Serum precipitant antibodies
CXR
HRCT
Lung function tests
Bronchoalevolar lavage
What may be seen on CXR in hypersensitivity pneumonitis?
Ground glass changes
Airspace consolidation
Interstitial opacities
What 5 features may be seen on HRCT in hypersensitivity pneumonitis?
Patchy, diffuse, symmetrical ground glass opacities
Small (<5mm) centrilobular opacities
Patchy air trapping on expiratory imaging
Airspace consolidation
Changes are typically bilateral in the mid-zone
Describe lung function tests in hypersensitivity pneumonitis
Acute: Restrictive pattern
Chronic: mixed pattern
TLCO reduced
What may be found on bronchoalveolar lavage in hypersensitivity pneumonitis?
Antibodies to causative antigen
Lymphocytosis
Describe first line management of hypersensitivity pneumonitis
Identification + removal of causative agent
How can corticosteroids be used in hypersensitivity pneumonitis?
Differentiate dx: HP tends to be steroid responsive, IPF does not
Symptomatic relief
Which patients with hypersensitivity pneumonitis may benefit from long term steroids?
Chronic HP may benefit from long term low-dose Prednisolone
What are the complications of hypersensitivity pneumonitis?
Fibrotic + emphysematous changes following inflammation may lead to fibrosis
May need LTOT
Reduced ability to perform ADLs
What is cryptogenic organising pneumonia (COP)?
diffuse ILD that affects distal bronchioles, respiratory bronchioles, alveolar ducts + alveolar walls
List 4 symptoms of COP
Cough
SOB
Fever
Malaise
What may be seen on bloods in COP?
Leukocytosis
High ESR + CRP
What is seen on imaging in COP?
CXR: Bilateral patchy infiltrates, diffuse consolidative or ground glass opacities
HRCT: triangle sign, air-bronchograms
What is the distinctive triangle sign commonly seen in COP?
Triangular ground glass opacity with the base on the pleura + apex towards the mediastinum
Describe lung function tests in COP
Classically restrictive pattern
(sometimes obstructive or normal)
TLCO reduced
Describe management of COP
Mild: watch + wait
Severe: high dose (40-60mg) Prednisolone PO
What is asbestosis?
Lung fibrosis related to asbestos exposure
What are pneumoconioses?
Group of lung diseases caused by inhalation of harmful dusts
(aka occupational ILDs)
Give 4 examples of pneumoconioses
Asbestosis
Silicosis
Coal workers pneumoconiosis (black lung disease)
Chronic beryllium disease (Berylliosis)
Give 2 features of silicosis on CXR
Progressive upper zone fibrosis
“Egg shell” calcification of the hilar LNs
Describe CXR in coal workers pneumoconiosis
Progressive upper zone fibrosis
Describe CXR in berylliosis
Progressive upper zone linear interstitial fibrosis
Describe lung function tests in pneumoconioses
May show restrictive, obstructive or normal pattern
Describe conservative management of pneumoconioses
Smoking cessation
Removal of occupational exposure
Advice regarding compensation
+/- pulmonary rehabilitation
What medication is used in berylliosis?
Prednisolone 40-70mg PO
