Interstitial Lung Disease Flashcards
Conditions affecting upper lobes
Silicosis, sarcoidosis Coal workers pneumoconiosis Histiocytosis Ankylosing spondylitis Allergic bronchopulmonary aspergillosis Radiation Tuberculosis
Conditions affecting lower lobes
Rheumatoid arthritis Asbestosis Scleroderma Cryptogenic fibrosing alveolitis Other - drugs - busulphan, nitrofurantoin, bleomycin, hydralazine, amiodarone
Radiological/histological classification of interstitial lung disease
Notes on idiopathic pulmonary fibrosis
- Chronic, progessive fibrosing lung disease, unknown cause
- Exclusion of other known causes for diagnosis e.g. CTD, drugs, occupational, environmental exposures
- UIP histologically (radiological UIP strong surrogate for this) - surgical biopsy rarely required
- Diagnosis at ILD-MDM mandatory
- Steroids increase mortality.
- In-hospital mortality for acute, non-infective exacberations = 50%
Risk factors
1. Older age
2. Male gender
3. Past smoking history
4. GORD
5. Family history ILD
6. Absence of alternative causes for iLD
Notes on treatment of idiopathic pulmonary fibrosis
Perfenidone
- Acts through TGF-B and reduces fibroblast proliferation
- Slows decline in FVC, improves survival
- Side effects: nausea, other upper GIT symptoms, photosensitive rash
Nintedanib
- Inhibits multiple tyrosine kinases (PDGF, VEGF, FGF)
- Slows decline in FVC
- Reduces incidence of acute exacberations, improves survival
- Side effects: diarrhoea, weight loss, possible cardiovascular risk, possible small increase in bleeding risk - avoid if on anticoagulants
Lung transplant - best treatment for selected patients
Pulmonary rehab effective in improving quality of life
Notes on lymphocytic interstitial pneumonia
- Associated with Sjogren’s syndrome, RA, SLE, myasthenia, chronic active hepatitis, HIV. Also anti-Ro, La antibodies
- Relatively good prognosis, treat underlying disease, steroids may be required
- CT: discrete peribronchovascular cysts, varying degrees of groundglass opacity
- Commonly mistaken for NSIP - need lung biopsy (overlap features with NSIP on CT)
Notes on non-specific interstitial pneumonia (NSIP)
- Tend to be younger, subacute onset, no clubbing
- Commmonly see bilateral basal predominant ground glass changes, without honeycombine or traction bronchiectasis, can get honeycombing in late stage of disease
- 2 pathologies -> cellular and fibrotic. Prognosis > cNSIP > fNSIP > UIP
- Seen in CTD - RA, SScl, PM/DM, Sjogren’s
- Treatment with immunosuppression, role for antifibrotics unclear
Notes on hypersensitivity pneumonitis
- Many causes, often very difficult to identify triggering agent - farmer’s lung, Finnish Sauna workers lung, maple bark disease, bagassosis, mushroom waorkers lng
- Upper zone predominance, fine nodules, starry night airway involvement. Inspiratory/expiratory scans -> air trapping, mosacism
- Good outcomes if trigger identified and removed - can progress to chronic HP with picture similar to IPF
- BAL helps, prednisone also used but poor evidence base
Notes on cryptogenic organising pneumonia
- CT: pleurally based, dense consolidation, triangular air bronchograms, peribronchovascular densities
- Aetiology: ?preceding pneumonia with persistent inflammatory response
- Treatment: steroids
- Good prognosis
Notes on pulmonary lymphangioleiomyomatosis
- Proliferation of abnormal smooth muscle
- Only seen in women, oestrogen dependent, aggravated by pregnancy
- Presents with dyspnoea, obstructive spirometry
- CT: diffuse bilateral cystic disease
- Causes: sporadic, associated with tuberous sclerosis
- Association with angiomyolipomas of the kidneys, and meningiomas
Notes on acute interstitial pneumonia
- Rare, usually fatal
- Diffuse alveolar damage, interstitial oedema, inflammatory cell infiltration, fibroblast proliferation and type II cell hyperplasia
- Hyaline membranes on biopsy
- AIP (idiopathic), or diffuse alveolar damage (non-idiopathic)
- HRCT: diffuse bilateral airspace changes
- Rx with BAL if fit enough
Notes on idiopathic pleuroparenchymal fibroelastosis
- Upper zone predominant
- Management supportive, slow progession
Notes on Langerhan’s cell hisstiocytosis
- Young adults 20-40
- Smokers - improves with smoking cessation
- M>F
- Multiple nodules
- Birbek or X granules on EM of lung biopsy
- Disease spares the lung bases
- 25% asymptomatic
- PTX in 10%
Notes on pulmonary alveolar proteinosis
- Deficiency of surfactant protein clearance -> protein builds up in lungs
- Mild inflammation can progess to fibrosis
- CT: diffuse bilateral ground glass changes. Crazy paving pathognomonic
- Treatment = whole lung lavage
- Good prognosis
List of eosinophilic lung diseases
- Acute eosinophilic pneumonia (and chronic)
- EGPA
- Loeffler’s syndrome
- ABPA
- Drug induced pneumonia
- Eosinophilic bronchitis
- Asthma
