This is an approach to management of Cryptogenic fibrosing alveolitis including diagnostic investigations .
This disease previously called Hamman Rich syndrome or Idiopathic pulmonary fibrosis .
Causes : Auto immune !
• It usually occurs in late middle age.
Clinical picture
- Picture of Interstitial pulmonary fibrosis (as before)
Investigations
* X-ray : Miliary shadows
* Respiratory function tests showing diffusion defect with restrictive hypoventilation.
* Blood gases : as before.
* CT scan lung, lung biopsy.
* BAL showing mainly alveolar macrophages.
* Transbronchial biopsy as before.
* Cytotoxic drugs may be added if there IS no response e.g azathioprine or cyclophosphamide.
This disease previously called Hamman Rich syndrome or Idiopathic pulmonary fibrosis .
Causes : Auto immune !
• It usually occurs in late middle age.
Clinical picture
- Picture of Interstitial pulmonary fibrosis (as before)
Investigations
* X-ray : Miliary shadows
* Respiratory function tests showing diffusion defect with restrictive hypoventilation.
* Blood gases : as before.
* CT scan lung, lung biopsy.
* BAL showing mainly alveolar macrophages.
* Transbronchial biopsy as before.
Treatment of Hamman Rich syndrome
* Steroids* Cytotoxic drugs may be added if there IS no response e.g azathioprine or cyclophosphamide.