Interstitial Lung Disease Flashcards
Definition
Umbrella term to describe conditions that effect the lung parenchyma causing inflammation and fibrosis.
Fibrosis definition
The replacement of the normal elastic and functional lung tissue with scar tissue is still and does not function effectively
Idiopathic Pulmonary Fibrosis
Progressive pulmonary fibrosis with no clear cause
S+S: Presents as insidious onset of SOB + dry cough for 3+ months
Epidemiology: + 50
Dx: Bibasal for inspiratory crackles + finger clubbing
Tx:
- Pirfenidone is an antifibrotic and anti-inflammatory
- Nintedanib is a monoclonal antibody targeting tyrosine kinase
Drug induced pulmonary fibrosis
Amiodarone
Cyclophosphamide
Methotrexate
Nitrofurantoin
Secondary Pulmonary Fibrosis
Pulmonary fibrosis can occur secondary to other conditions:
- Alpha-1 antitripsin deficiency
- Rheumatoid arthritis
- Systemic lupus erythematosus (SLE)
- Systemic sclerosis
Hypersensitivity Pneumonitis (AKA Extrinsic Allergic Alveolitis)
Type 3 hypersensitivity reaction to an environmental allergen that causes parenchymal inflammation and destruction in people that are sensitive to that allergen.
Dx = Bronchoalveolar lavage = raised lymphocytes and mast cells in hypersensitivity pneumonitis
Tx = removing the allergen + giving O2 where necessary + steroids
Examples of specific causes:
- Bird-fanciers lung is a reaction to bird droppings
- Farmers lung is a reaction to mouldy spores in hay
- Mushroom workers’ lung is a reaction to specific mushroom antigens
- Malt workers lung is a reaction to mould on barley
Cryptogenic Organising Pneumonia
Cryptogenic organising pneumonia = Focal area of inflammation of the lung tissue.
Aetiology: Idiopathic or triggered by infection, inflammatory disorders, medications, radiation or environmental toxins or allergens.
Px: V.similar to infectious pneumonia = SOB, cough, fever and lethargy.
Similarly to pneumonia on a chest xray with a focal consolidation.
Dx: Often delayed due to the similarities to infective pneumonia. Lung biopsy is the definitive investigation.
Tx: systemic corticosteroids.
Asbetosis
Asbestosis is lung fibrosis related to the inhalation of asbestos. Asbestos is fibrogenic + oncogenic, (causes cancer). The effects of asbestos usually take several decades to develop. Asbestos inhalation causes several problems:
- Lung fibrosis
- Pleural thickening and pleural plaques
- Adenocarcinoma
- Mesothelioma
Suffers are eligible for compensation if they develop asbestos related health conditions (except isolated pleural plaques). All patients that die with known exposure to asbestos need to be referred to the coroners .
Granulomatous
Sarcoidosis
Inhalation
Hypersensitivity pneumonitis
Pneumoconiosis (asbestosis/silicosis)
Conns tissue
Scleroderma
R.A
Other
Good pastures
Vasculitis
Diagnosis
Combination of clinical features and high resolution CT scan of the thorax
- ground glass appearance
GOLD STANDARD = lung biopsy
Management
Irreversible damage
Supportive treatment:
- Remove or treat the underlying cause
- Home oxygen where they are hypoxic at rest
- Stop smoking
- Physiotherapy and pulmonary rehabilitation
- Pneumococcal and flu vaccine
- Advanced care planning and palliative care where appropriate
- Lung transplant
