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Interstitial lung disease (Complications (Malignancy Bronchial ca …
Interstitial lung disease
Definition
Umbrella term for a number
of conditions affecting the
lung parenchyma
Pathophysiology
Chronic inflammation
Over time, fibrosis and
remodelling of parenchyma
Interstitium, bronchi, bronchioles and alveoli
Clinical
presentation
SOB
(worse on exertion)
Cough
(mostly non-productive;
black sputum if coal dust)
Weight loss
Malaise
Types
Systemic disease
Sarcoidosis
Vasculitis e.g. Chrug-Strauss, Wegener's
RA, SLE, systemic sclerosis, Sjogren's, etc.
Idiopathic
Most common cause
Inflammation trigger by unknown cause
Irritant
Occupational
Asbestos (building, roofing)
Silicone (mining, stone quarry, pottery/ceramics, sandblasting)
coal dust (coal mining)
Drugs
Abx - nitrofurantoin
Chemo - bleomycin, busulfan
Immunosupp - sulfasalazine
Amiodarone
Hypersensitivity/EAA
Infectious agents - mushroom and sugar workers [spores],
malt workers [aspergillus]
Proteins - bird fanciers lung [bird droppings],
farmers lung [hay, mould]
Infection
Viral, fungal, TB
GORD
Acid irritation over time
Diagnosis
Examination
Clubbing, reduced chest expansion
Abnormal breath sounds, coarse/fine creps
Investigations
Bedside
Obs, ECG
Bloods
FBC (may have high WCC), CRP/ESR
ABG (hypoxia, hypercapnia)
Hypersensitivity tests
Immunology - ACE (sarcoid), ANCA (vasculitis), ANA (SLE) etc.
Imaging
CXR - ground glass (idiopathic), diffuse reticular lines,
round opacities, military/nodular pattern,
consolidation/shadows, upper zone mass (PMF)
CT thorax - honeycombing, irregular opacities
Special tests
LFTs - restrictive
Gas transfer - reduced
Biopsy
Exclude other conditions
History
PMH - systemic disease
SH - industrial exposure, occupation
Management
Initial ABCDE
Definitive
Conservative
Avoid further exposure to irritants
Compensation (asbestos, silicone, coal, etc)
Pulmonary rehabilitation
Medical
Steroids
Indication: acute HAA, acute sarcoidosis
E.g. initial PO prednisolone then
long-term steroid
Immunosuppressants
Indication: severe sarcoidosis, vasculitis
Anti-inflammatories
Indication: acute sarcoidosis
E.g. NSAIDs
Immunotherapy
Refractory sarcoidosis, vasculitis
E.g. anti-TNFa, anti-CD20
Analgesia
Opiates
Oxygen therapy
Surgical
Lung transplant
Complications
Malignancy
Bronchial ca
Mesothelioma
Infection
TB (silicosis)
Progressive massive fibrosis (PMF)
Coal workers lung
Progressive SOB, fibrosis, cor pulmonal, resp failure
Cor pulmonale
T1 resp failure
Pulmonary HTN