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Cryptogenic fibrosing alveolitis (Hamman Rich $) causes, diagnosis, treatment, prognosis

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)
Cryptogenic-Fibrosing-Alveolitis-x-ray

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.

Prognosis:

 * The median survival time for patients is about 5 years.
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Tamer Mobarak

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