Interstitial Lung Disease Flashcards
What is interstitial lung disease?
An umbrella term to describe diseases of the lung parenchyma
What does ILD cause aetiologically?
Inflammation and irreversible fibrosis
Name 6 different ILDs.
- Idiopathic pulmonary fibrosis (PF)
- Drug induced PF
- Secondary PF
- Hypersensitive pneumonitis
- Cryptogenic organising pneumonia
- Asbestosis
What is the prognosis of ILD?
Very poor- some as short as ~a year
Why is ILD a restrictive pattern disease?
Reduces lung compliance
What is idiopathic PF?
Idiopathic fibrosis of the lung parenchyma (most common ILD)
- Adults over 50
- Prognosis 2-5 years
Which drugs cause drug-induced PF?
CMAN
- Cyclophosphamide
- Methotrexate
- Amiodarone
- Nitrofurantoin
What causes secondary PF?
Rheumatological diseases (association with ANA primarily and RF)
- SLE
- Systemic sclerosis
- RA
- Alpha 1 anti-trypsin deficiency
- Sjogrens
What is hypersensitivity pneumonitis?
Type 3 hypersensitivity reaction to an allergen
Which allergens can cause hypersensitivity pneumonitis?
- Bird droppings
- Cotton
- Mould
- Mushrooms
What is bronchoalveolar lavage?
Method of collecting cells from the airways:
1- Wash the airways with fluid
2- Collect that fluid back for testing
What does bronchoalveolar lavage show in hypersensitivity pneumonitis?
Raised lymphocytes and mast cells
What is cryptogenic organising pneumonia?
Focal area of inflammation in the lung
What can cause cryptogenic organising pneumonia?
- Idiopathic
- Infection
- Inflammatory disorders
- Medications
- Radiation
- Environmental toxins and allergens
What is asbestosis?
Lung fibrosis related to asbestos inhalation- asbestos is fibrogenic
What four problems can asbestos cause?
- Asbestosis (PF)
- Pleural thickening and plaques
- Adenocarcinoma
- Mesothelioma
What does PF look like on a HRCT?
Ground glass
How does PF present?
- Dyspnoea
- Dry cough
- Fatigue
How does cryptogenic organising pneumonia present?
Like pneumonia:
- Fever
- Lethargy/fatigue
- Cough
- Dyspnoea
- Chest pain
What examination findings would you see for an ILD?
- BIBASAL fine end inspiratory crackles
- Clubbing
What will lung function tests show?
Restrictive pattern:
- Both FVC and FEV1 less than 80%
- But ration still over 0.7
What support should patients be offered?
- Advanced care planning
- Pulmonary rehabilitation
- Annual flu and 5 yearly pneumococcal vaccine
What specific treatment for the diseases is there?
- Idiopathic PF- Pirfenidone (antifibrotic) or Nintendanib (mAB to tyrosine kinase)
- Hypersensitivity pneumonitis- remove allergen, give steroids
- Cryptogenic organising pneumonia- corticosteroids
What surgery is used in some cases?
Transplant
What type of respiratory failure do ILDs cause?
Type 2
