Interstitial Lung Disease
Group of diseases
Med by immune mechanisms
Interstitial inflammation & fibrosis
Defined by lung function tests
Restrictive pattern (reduced lung compliancy & gas exchange)
Causes
Pneumoconiosis
Systemic CT (autoimm) disease
Sarcoidosis
Wegener's granulomatosis
Goodpasture's Syndrome
Post-RTx/CTx
Post ARDS
Idiopathic pulmonary fibrosis
Sarcoidosis
Systemic disease of unknown aetiology
F>M
Black>>White>>Asian
Prob response to Ag
Activated T-Cells & macrophages
Hilar LN/lung involvement in 90%
Skin/LN/Kidney involvement*
Raised serum ACE
*High calcium
Pathology
Granulomatous inflammation w/o necrosis
Idiopathic Pulmonary Fibrosis
aka
cryptogenic fibrosing alveolitis/usual interstitial pneumonitis/Hamman-Rich syndrome when rapidly progressive
M>F
60+ yrs
Interstitial pneumonitis & fibrosis
?cause
Pathology
End stage 'Honeycomb' lung (cystic air-spaces from interstitial fibrosis)
Extensive interstitial fibrosis with alveolar distortion & interstitial expansion
Tx
Steroid/further imm-supp
Variable response
*Progress to significant fibrosis
Cx
Respiratory failure
Cor pulmonale
Pulmonary HT
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