Transcript What the GP should know about fibrosing alveolitis

What the GP should know
about ‘Fibrosing alveolitis’
Dr Paul Beirne
‘Fibrosing Alveolitis’
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What is it?
Symptoms
Signs
History taking
Screening tests
Securing a diagnosis
Prognosis and treatment
Interstitial Lung Diseases
Known cause
e.g. drugs
OR
Known association
e.g. CTDs
Idiopathic interstitial
pneumonia
IPF
Granulomatous
e.g. Sarcoid, EAA
Others
(Eosinophilic pneumonia,
LAM, Histiocytosis X,
Alveolar proteinosis,
Cancer)
Other
COP
NSIP
DIP/RBILD
LIP
AIP
IPF = Idiopathic Pulmonary Fibrosis (Cryptogenic Fibrosing Alveolitis (CFA))
Symptoms
• Dyspnoea
• Cough
• Constitutional
• None
Signs
• Digital clubbing 25-50%
• Bibasilar fine late inspiratory crackles (‘Velcro’
crackles)
• Look for:
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Cyanosis
Cor pulmonale (raised JVP, peripheral oedema)
Rheumatological signs
Evidence of malignancy
History
• Drugs
• Occupational
– Asbestos
– Silica or coal dust
– Metals
• Environmental
– Smoking
– Birds, moulds/damp (e.g. compost, hay)
• Family
• Rheumatological
– Arthralgia
– Raynauds
– Dry eyes or mouth
Screening Tests
• Spirometry
• CXR
Spirometry in Pulmonary Fibrosis
• Restrictive
– FVC greatly reduced
– FEV1 reduced
– FEV1/FVC ratio
increased to > 80%
• Pitfalls
– Smokers
– Sarcoidosis etc.
CXR
• Normal
• Peripheral basal reticular shadowing suggests
Idiopathic Pulmonary Fibrosis
• Other findings in other pulmonary fibrotic
diseases include
– reticulonodular shadowing
– ground glass shadowing
– consolidation
• Accurate diagnosis on CXR alone is not possible
Securing a diagnosis
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History
Examination
HRCT chest
Lung function
– Spirometry
– Lung volumes
– Gas transfer
• Bronchoscopy
– Bronchoalveolar lavage
– Transbronchial lung biopsies
• Surgical lung biopsy???
– VATS
Prognosis
• Idiopathic Pulmonary Fibrosis
– Inexorable slow decline punctuated by
periods of accelerated decline
– Median time from diagnosis to death 2.5
years
• Non-Specific Interstitial Pneumonitis
(NSIP)
– Variable prognosis with recovery or stability
on treatment being usual; minority progress to
death
Treatment
• Steroids
• Steroid sparing agents
– Azathioprine
– Mycophenolate
• Antioxidants
– NAC
• For Idiopathic Pulmonary Fibrosis, current best
treatment:
– NONE
– ‘Triple therapy’ with low dose prednisolone, azathioprine 2 mg/kg
and NAC
– Early referral for transplant
Summary
• Pulmonary fibrosis thickens the alveolar wall and
impairs gas exchange
• Presents with dyspnoea and cough
• Commonest signs are clubbing and crackles
• Spirometry often restrictive
• CXR usually abnormal
• Accurate diagnosis in secondary care is
essential as prognosis and treatment vary
markedly between different diseases