Interstitial Lung Disease - Sarcoidosis Flashcards
What is interstitial lung disease?
Examples
Umbrella term describing a number of conditions that cause inflammation + fibrosis the lung parenchyma:
- usual interstitial pneumonia (including. Idiopathic pulmonary fibrosis)
- non-specific interstitial pneumonia
- hypersensitivity pneumonitis
- sarcoidosis
- asbestosis
Presentation of interstitial lung disease
dyspnoea on exertion
dry cough
fatigue
Diagnosis of interstitial lung disease
- clinical features
- high-resolution CT scan of thorax: ground glass appearance
- spirometry >70%
- lung biopsy
- bronchoalveolar lavage
What blood tests should be done after a diagnosis of interstitial lung disease for diseases associated with ILD?
- ANA: SLE
- Rheumatoid factor + anti-CCP: RA
- ANCA: vasculitis
- Anti-GBM: pulmonary renal disease
- ACE: sarcoidosis
- HIV test
What is the most common type of pulmonary fibrosis?
Usual interstitial pneumonia (incl. idiopathic pulmonary fibrosis)
Typical findings of idiopathic pulmonary fibrosis
- progressive exertional dyspnoea
- dry cough
- basal fine end-inspiratory crackles
- finger clubbing
- reduced chest expansion
- pulmonary fibrosis
What is hypersensitivity pneumonitis?
inflammation of lungs due to immune response to inhaled organic antigens or chemicals e.g. mould, dust, animal proteins
Types of presentation of hypersensitivity pneumonitis
- acute : 4-8 hours after exposure, often reversible + spontaneous settles after 1-3 days
- chronic: chronic exposure + less reversible
Common drug causes of pulmonary fibrosis
- amiodarone
- methotrexate
- nitrofurantoin
- penicillamine
- bleomycin
What is sarcoidosis?
Multisystem inflammatory disease characterised by formation of non caseating granulomas (no necrosis)
Where is sarcoidosis often found?
lungs
lymph nodes
Typical demographic of sarcoidosis
- 20-40 or ~60 years old
- woman
- black ethnic origin
Presentation of sarcoidosis
- 20-40 year old black woman
- shortness of breath
- dry cough
- erythema nodosum
- weight loss
- fatigue
- uveitis, conjunctivitis
- polyarthralgia
- lupus pernio
- hypercalcaemia stones, groans, moans + bones
Blood test findings of sarcoidosis
raised ACE + calcium
Skin findings of sarcoidosis
- erythema nodosum: raised, red, painful, tender nodules across both skins
- lupus pernio: raised purple skin lesions often on cheeks + nose
What is Lofgren’s syndrome?
What is it related to?
- erythema nodosum
- bilateral hilar lymphadenopathy
- polyarthralgia
Sarcoidosis
What is asbestosis?
lung fibrosis related to asbestos exposure
What can asbestos inhalation cause?
- lung fibrosis
- pleural thickening + plaques
- adenocarcinoma
- mesothelioma
Management + treatment of interstitial lung disease
- pirfenidone + nintendanib to slow progression
- remove exposure
- avoid associated drugs
- smoking cessation
- treatment of infective exacerbations
- annual flu + pneumococcal vaccines
- physiotherapy + pulmonary rehabilitation
- O2 if respiratory failure
- advanced care planning+ palliative care
- lung transplant
What two drugs are used to slow progression of ILD?
pifenidone
nintedanib
Spirometry findings of idiopathic pulmonary fibrosis
FEV1 normal/reduced
FVC decreased
FEV1/FVC >70%
Reduced TLCO
Imaging findings of sarcoidosis
- CXR: hilar lymphadenopathy
- high resolution CT: hilar lymphadenopathy + pulmonary nodules
Management of sarcoidosis
- conservative in most patients
- oral steroids first line + bisphosphonates
- methotrexate second line
Fibrosis mainly affecting upper zones -
- hypersensitivity penumonitis
- sarcoidosis
- tuberculosis
- radiation induced pulmonary fibrosis
- ankylosing spondylitis (rare)
