Interstitial Lung Disease Flashcards
What is the new name for interstitial lung disease?
Diffuse Parenchymal Lung Disease
How are interstitial lung diseases classed?
Idiopathic if the cause is not known
Classified according to cause if known
What are some known causes of interstitial lung diseases?
Connective tissue diseases- SLE, RA, Systemic sclerosis
Occupational exposures- Asbestosis, Silicosis
Granulomatous- Sarcoidosis, Hypersensitivity pneumonitis
Treatment induces- e.g. Amiodarone, Methotrexate, Nitrofurantoin, Bleomycin
What are some of the idiopathic types of interstitial lung disease?
Idiopathic pulmonary fibrosis
Cryptogenic organizing pneumoni
What is main approach when investigated interstitial lung diseases?
Must rule out interstitial lung disease due to known causes as these are potentially treatable
How do interstitial lung diseases often present?
Shortness of breath
Worsens on exertion
Dry cough
What is heart on auscultation for interstitial lung diseases?
Fine crackes
What occupations would you consider for the different causes of ILD?
Asbestosis- Builder
Berylliosis- Aircraft engineer
Silicosis- Sand blaster
Hypersensitivity pneumonitis- Farmer, Bird keeper
What might be a cause of ILD in a rheumatoid arthritis patient?
The RA itself
Or the methotrexate being used to treat it
What might be seen on auscultation?
Clubbing
Fine crackles
RHF signs
What might you think of in a patient with bluish discolouration of the skin and ILD?
Amiodarone induced fibrosis
What are some features of sarcoidosis?
ILD Bilateral hilar lymphadenopathy Eryhthema nodosum Hypercalcaemia Uveitis/Conjunctivitis Swelling of the parotids
What investigations should be done for ILD?
CXR- Reticular infiltrates HRCT of thorax- shows characteristic honeycombing Spirometry- Restrictive pattern TLCO- Reduced transfer factor Bronchoalveolar Lavage Lung biopsy to confirm diagnosis
Other investigations will depend upon the cause:
Drug review, ANTI- CCP, ANA, Anti- SM, ESR, CRP, Anti Jo-1 (polymyositis/dermatomyositis). Pleural effusions may also be seen in connective tissue diseases.
What spirometry changes are seen in ILD?
Restrictive pattern: FVC decreased FEV1 normal FEV1: FVC >0.7 TLC goes down
What test measures the barriers to diffusion in the lung?
TLCO
There is a reduced transfer factor in ILD
What are some of the causes of upper lobe fibrosis? (Use mnemonic)
Coal workers Hypersensitivity Histiocytosis Allergic bronchopulmonary aspergilliosis Anklyosing spondlyitis Radiation Tuberculosis Silicosis
CHHAARTS- Top of the charts = Upper lobe
What are some of the cause of lower lobe fibrosis? Use mnemonic
Rhemuatoid arthritis Asbesosis Idiopthic Pulmonary Fibrosis Scleroderma Exogenous drugs
What is elevated in sarcoidosis?
ACE
Calcium
This is because the granulomas secrete vitamin D.
Other features- Bilateral hilar lymphadenopathy, Erythema nodosum
What infective disease causes exertional dyspnoea and dry cough?
PJP
Sample with induced sputum, bronchoalveolar lavage
Why might a bronchoalveolar lavage be done in ILD?
Hypersensitivity pneumonitis and sarcoidosis both show raised lymphocytes on BAL.
What causes extrinsic allergic alveolitis? Who is it seen in?
Exposure to allergens in the airways resulting in a hypersensitivity reaction. This is hypersensitivity pneumonitis.
It is seen in: Bird keepers- Bird fanciers lung Farm workers Mushroom workers (fungal allergen) Sugar workers
What is the treatment for ILD?
If causative- manage appropriately, avoid exposure to allergens if hypersensitivity pneumonitis, stop amiodarone/methotrexate/chemotherapy agents, immunosuppression for connective tissue disease, avoid occupational expsoures
Oxygen therapy Stop smoking Antifibrotic agents being trialed Steroids for severe hypersensitivity reactions Lung Transplantation
Do patients always require treatment for sarcoidosis?
Not if asymptomatic
If symptomatic steroids, azathioprine, cyclophosphamide
What are two antifibrotic agents being used for idiopathic pulmonary fibrosis?
Nintedanib- Tyrosine kinase inhibitor
Pirfenidone- Regulates TGF Beta.
What are two complications of asbestos exposure?
Asbestosis
Mesothelioma
What type of granulomas are seen in sarcoidosis
Non-caseating
How is acute sarcoidosis treated?
Most resolve
Bed rest and NSAIDs
If don’t resolve steroids and then steroid sparing agents such as cyclosporine and azathioprine
