Interstitial lung disease Flashcards
What causes fibrosis in the upper zones?
CHARTS Coal worker's pneumoconiosis Hypersensitivity pneumonitis Ankylosing spondylitis Radiation TB Sarcoidosis/silicosis
What causes apical fibrosis?
Ank spond
What causes lower zone fibrosis?
IRA SAD Idiopathic pulmonary fibrosis RA Aspiration pneumonia Scleroderma Asbestosis Drugs
What is sarcoidosis?
Multisystem granulomatous disorder of unknown cause
Characterised by non caseating granulomas
Who is sarcoidosis seen in?
20-40yo
Women
Afro-Caribbean more
What is the presentation of sarcoidosis?
Acute= fever, erythema nodosum, polyarthralgia, bilateral hilar lymphadenopathy Non productive cough, dyspnoea, wheeze Chronic fatigue, weight loss Arthralgia Lymphadenopathy Uveitis
What investigations are done for sarcoidosis?
CXR Bloods- FB, U&Es, LFTs, Ca Lung function tests Biopsy ECG
What is seen on CXR with sarcoidosis?
Bilateral hilar lymphadenopathy
Pulmonary fibrosis- upper zone dominance
Displaced hilum
What is the management of sarcoidosis?
Corticosteroids if symptomatic, eye, heart or near involvement, or hypercalcaemia
What is usual interstitial pneumonitis?
Umbrella term for progressive fibrosis and chronic inflammation of interstitium
What can cause usual interstitial pneumonitis?
Idiopathic Connective tissue disease- usually RA Drug induced pulmonary fibrosis Post infection Industrial exposure- asbestosis, silicosis
What drugs can cause drug induced pulmonary fibrosis?
Amiodarone Methotrexate Sulfasalazine Cyclophosphamide Nitrofurantoin
What is the presentation of asbestosis?
SOB, dry cough
Reduced exercise tolerance
Lower lobe fibrosis
Associated with mesothelioma
What investigations are done for usual interstitial pneumonitis?
CXR
CT
What is the appearance of UIP on CXR?
Honeycomb damage of peripheries and lower portions of lungs
What is the management of UIP?
Oxygen therapy
Lung transplant
What is the prognosis for UIP?
Very variable
Generally 3-5years
What is idiopathic pulmonary fibrosis?
Excessive collagen deposition in pulmonary interstitium
Who is idiopathic pulmonary fibrosis seen in?
60-70yo
Men > women
What is the presentation of idiopathic pulmonary fibrosis?
Insidious onset SOB Dry cough Clubbing Bibasal inspiratory crackles Weight loss
What investigations are done for idiopathic pulmonary fibrosis?
CT= imaging of choice
CXR
Spirometry
Biopsy
What is the appearance of idiopathic pulmonary fibrosis on CXR?
Bilateral interstitial shadowing
Ground glass appearance –> honeycomb
What is the management of idiopathic pulmonary fibrosis?
Antifibrotic
Lung transplant
Pulmonary rehab
What is the prognosis for idiopathic pulmonary fibrosis?
Poor
What is hypersensitivity pneumonitis?
Inflammation of alveoli caused by type III and IV hypersensitivity reactions
What are some examples of hypersensitivity pneumonitis?
Bird fancier’s lung
Farmer’s lung
What is the presentation of hypersensitivity pneumonitis?
Acute= 4-8 hours after exposure -SOB, dry cough, fever Chronic -SOB, cough, crackles, wheeze -low grade illness
What investigations are dene for hypersensitivity pneumonitis?
FBC, CRP, ESR= no eosinophilia
CXR= upper/mid zone fibrosis
Bronchoalveolar lavage= raised lymphocytes and mast cells
What is the management of hypersensitivity pneumonitis?
Avoid allergen
Corticosteroids
What is the presentation of pulmonary eosinophilia?
Cough
Weight loss
Night sweats
What investigations are done for pulmonary eosinophilia?
CXR
FBC= high eosinophils
Bronchoalveolar lavage= eosinophilia
What is the management of pulmonary eosinophilia?
Steroids
What is the presentation of allergic bronchopulmonary aspergillosis?
SOB, wheeze, cough
Proximal bronchiectasis
Previous diagnosis of asthma
What investigations are done for allergic bronchopulmonary aspergillosis?
FBC, IgA= raised
CXR
Aspergillus= +
What is the management of allergic bronchopulmonary aspergillosis?
Steroids
What are the stages of sarcoidosis?
1= bilat hilar lymphadenopathy 2= BHL + interstitial infiltrates 3= diffuse interstitial infiltrates 4= diffuse fibrosis
