Case 5 - Interstitial Lung disease Flashcards
What are the 4 main types of interstitial lung disease?
- Usual intersitial pnuemonia (UIP)
- Non-specific intersitial pneumonia (NSIP)
- Extrinsic allergic alveolitis
- Sarcoidosis
What is important to ask about in the history for ILD?
- Occupation and environmental history - did they have exposure to asbestos (asbestosis), dust (silicosis) or coal (pneumoconiosis)
Pattern seen on pulmonary function tests for ILD
Restrictive
What investigations should be done for any new ILD diagnosis?
See if have any of the conditions associated with development of ILD including:
* ANA - CT disease OR SLE
* ENA - CT
* Rh F - RA
* ANCA - vasculitis
* Anti GBM - pulmonary renal disease
* ACE - sarcoidosis
* IgG to serum precipitins eg pigeon, budgie - can check for extrinsic allergic alveolitis
* HIV
Most common type of ILD
Usual interstitial pneumonia (UIP) - was known as idiopathic pulmonary fibrosis previously
Classical examination findings for UIP
- Clubbing
- Reduced chest expansion
- Fine inspiratory crepitations (like velcro - usually best heard basal/axillary areas)
- Pulmonary HTN features?
Cause of extrisnic allergic alveolitis
- AKA hypersensitivity pneumonitis
- Inhalation of organic antigen to which the individual has been sensitised
Presentation of EAA
- ACUTE - short period from exposure, 4-8hrs, usually reversible, spontaneously settle in 1-3 days, can recurr
- Or CHRONIC - chronic exposure (months-years), less reversible
Common drugs that can cause allergic alveolitis
- Amiodarone
- Bleomycin
- Methotrexate
- Nitrofurantoin
- Penicillamine
What is sarcoidosis?
- Multisystem inflammatory condition of unknown cause
- Commonly involves resp system but can affect nearly all organs
- Immunological response
- 50% –> spontaneous remission, others –> progressive
Histology of sarcoidosis
- Non-caseating granuloma
Simple investigations for sarcoidosis
- Pulmonary function tests
- CXR - can stage 1-4
- Urinary calcium
Bloods for sarcoidosis
- Renal function
- ACE
- Calcium
- LFTs
- ECG
Cardiac tests for sarcoidosis
- ECG
- 24hr ECG
- ECHO
- Cardiac MRI
Sarcoidosis neurological investigation
- MRI head (NOT CT) if headaches present
- Could be neurosarcoid
ILD treatment principles
- Depends on underlying cause/pathology
- Occupational/enviromental exposure - remove
- Avoid drug if caused by this
- Stop smoking
- MDT approach
- Treat infective exacerbations
- O2 if resp failure
- Palliative care
- Transplantation?
Specialist medication for ILD
- Pirfenidone - slows progression, anti-fibrotic assessed via MDT for this
Symptoms of ILD
- SOB
- Non-productive cough
- Fatigue
- Mild pain in chest
- Decreased appetite
Clinical signs of ILD
- Clubbing
- Widespread inspiratory crackles - velcro like
- Decreased chest expansion
CXR sign for ILD
- Alveolar shadowing/reticulonodular pattern of consolidation
- Unclear cardiac borders
Spirometry results for restrictive pattern
FEV1/FVC - normal, >0.7
FEV1 - normal/decreased
FVC - decreased
Cause of alveolar shadowing in CXR
- Parenchyma disease
- Cotton wool like appearance
What is definitive test which confirms ILD?
High resolution CT - honeycombing
Difference between UIP and NSIP?
Different patten on CT scan
