INTERSTITIAL LUNG DISEASE Flashcards
Define interstitial lung disease.
Chronic inflammation leading to fibrosis of the lung parenchyma. This reduces compliance of the lung tissue and hence leads to difficulty breathing.
In ILD, is it the alveoli or the airways that are affected?
Alveoli. The disease may involve the alveoli themselves, the interstitial space or the capillaries.
What is the most common ILD?
Usual interstitial pneumonitis (UIP)
What are most acute episodes of parenchymal inflammation the result of?
Hypersensitivity to a number of possible allergens. Eg birds.
What pattern will be seen in lung function tests with someone with ILD?
A restrictive pattern.
What is a restrictive pattern in a lung function test?
Normal or even increased FEV1/FVC ration but a reduced FEV1 and FVC.
Will the diffusing capacity (DLCO) and the (KCO) be affected in a patient with ILD?
Yes. They will both be reduced.
What inhaled substances often associated with a profession may lead to someone developing ILD? (Name 3)
Asbestos
Crystalline Silica
Beryllium
Which classes of drug can lead to a patient developing ILD? Give an example of a specific drug for each class. (Name 5 classes)
Antibiotics - Nitrofurantoin
Chemotherapeutic drugs - Methotraxate, Bleomycin, Busulfan
Anti-arrythmics - Amiodarone
Some drugs used to treat rheumatoid arthritis - Sulfasalazine
Statins - Simvastatin (more than atorvastatin)
Which connective tissue disorders are associated with ILD? (Name at least 4, there are 6 given)
Rheumatoid arthritis Systemic Lupus Erythematosus (SLE) Sjorgen's syndrome Systemic sclerosis Polymyositosis Dermatomyositosis
Which infections are recognised as being risk factors for developing ILD? (Name all 5)
Tuberculosis Chlamydia Trachomatis Respiratory syncytial virus (RSV) Atypical pneumonias Pneumocystic pneumonias
Name 4 interstitial lung diseases that are considered idiopathic.
Hamman-Rich syndrome (also called Acute Interstitial Pneumonitis)
Idiopathic Pulmonary Fibrosis
Cryptogenic Organising Pneumonitis
Lymphocytic Interstitial Pneumonitis
Sarcoidosis - not classically considered an ILD but the presenting picture is so similar
How would a patient with suspected ILD normally present? (Name 2 symptoms)
Dyspnoea on exertion
Paroxysmal (suddenly getting worse) non-productive cough
Weight loss
Malaise
On examination what might you find in a patient with suspected ILD? (Name 2 signs)
Cyanosis
Abnormal breath sounds - fine end inspiratory crepitations
Clubbing
O2 sats may also be low
What investigations would be needed to confirm a diagnosis of ILD?
Chest x-ray
Chest CT
Lung function test
What are you likely to see on a chest x-ray of someone with ILD?
Widespread or local areas of haziness
What are you likely to see on a chest CT of someone with ILD?
A CT could reveal areas of ground glass or honeycombing. This might help to pinpoint a more specific diagnosis based on the pattern of disease.
Can idiopathic pulmonary fibrosis be reversed and cured?
No. Treatment is focused on reducing symptoms, stopping disease progression, preventing acute exacerbations and prolonging survival.
Is someone with idiopathic pulmonary fibrosis more likely to develop type 1 or type 2 respiratory failure?
Type 1
What are the possible complications of idiopathic pulmonary fibrosis?
Type 1 respiratory failure
Heart failure
Infection
Lung cancer
What are the treatment options for someone with idiopathic pulmonary fibrosis (IPF)? (Name 5 things that can be done to manage the disease)
Pirfenidone - an anti-inflammatory, anti-oxidant and anti-fibrotic agent. It prevents fibroblast proliferation. Some evidence to suggest is may halt disease progression in mild cases.
Long term oxygen therapy (LTOT)
Opiates - considered for those with severe IPF (often part of palliative care)
Vaccinations - preventing further infections is the best way to prevent disease progression.
Smoking cessation
Pulmonary physiotherapy and exercise
Single lung transplant - in those patients that are fit enough to survive major operation.
