Related Subjects:
|Idiopathic Pulmonary Fibrosis
|Diffuse Parenchymal Lung disease
|Asbestos Related Lung disease
|Sarcoidosis
|Coal Worker's Pneumoconiosis
|Silicosis
|Farmer's Lung
|Cryptogenic Organising Pneumonia (COP-BOOP)
|Extrinsic Allergic alveolitis (Hypersensitivity)
|Byssinosis
|Pneumoconiosis
|Cor Pulmonale
About
- Also called Diffuse Interstitial Lung Disease
- Can be due to over 200 conditions
- Inflammation and fibrosis of the pulmonary interstitial and alveolar space
Aetiology
- Many of these entities are of unknown cause and little is understood about their pathogenesis.
- MUC5B genotype, associated with a mild form of IPF
- Smoking increases risk as do occupational exposure
Known Types
- Idiopathic pulmonary fibrosis
- Sarcoidosis
- Connective tissue disease-associated ILD
- Hypersensitivity pneumonitis
- Farmer's Lung
- Bird Fancier's lung
- Cryptogenic organizing pneumonia.
- Secondary to
- Rheumatoid arthritis
- Scleroderma (systemic sclerosis)
- Polymyositis and dermatomyositis
- Inflammatory bowel disease
- It may also be caused by exposure to drugs, such as:
- Chemotherapy agents - Methotrexate, Bleomycin
- Antiarrhythmics
- Statins
- Antibiotics
- Pneumoconiosis
- Asbestos, Silica
- Coal dust
Clinical
- Progressive breathlessness and cough
- Reduced chest expansion
- Weight loss, end inspiratory fine velcro like crackles
- Progressive disease and respiratory failure
- Clubbing - seen with IPF and Asbestosis
- Swollen joints, synovitis, rashes, erythema nodosum
- Haemoptysis and chest pain and sputum not common
- Associated conditions: rash, neurology, joint disease
Investigations
- FBC, U&E, CRP, ESR, RF, ANA
- CXR: reticular changes like a net
- ACE: tends to be unhelpful but tested with Sarcoid.
- BNP if CCF expected
- High-resolution computed tomography (HRCT) scanning: reticular or ground-glass abnormalities, diffuse centrilobular nodularity, non-emphysematous cysts, honeycombing or traction bronchiectasis, subpleural changes
- Spirometry: Low Fev1 and FVC and DLCO
- Transbronchial biopsy : pathologists can identify a pattern of usual interstitial pneumonia with high confidence.
- Surgical biopsy may be performed to help diagnose your condition
- ANA : proteins against nuclear cells
- Extractable ENA: Looks for specific abs
- Rheumatoid factors anti CCP
- Dermatomyositis: Anti Jo-1
- Scleroderma SCL70
- RAST test to allergens for hypersensitivity pneumonitis
Differential
- Bronchiectasis
- Heart failure
Pathology
- Honeycomb lung appearance
- In some cases, Inflammatory cells (granuloma), as is seen in sarcoidosis.
- Upper lobe: SCHART: Silicosis/Sarcoid/Coal Workers, Langan Histiocytosis, TB, radiation, Ankylosing spondylitis
- Lower Lobe: RASIO: RA, Asbestosis, Scleroderma, IPF, Other
Management
- Supportive with Oxygen therapy
- Current management strategies for the more commonly encountered ILDs including idiopathic pulmonary fibrosis, sarcoidosis, connective tissue disease-associated ILD and hypersensitivity pneumonitis
References