Skip to
  1. Homepage
  2. Rare diseases
  3. Search
PrintPrint
Simple search

Simple search

*
(*) mandatory field





Other search option(s)

Cryptogenic organizing pneumonia

Orpha number ORPHA1302
Prevalence of rare diseases Unknown
Inheritance
  • Sporadic
Age of onset Adulthood
ICD 10 code
  • J84.8
MIM number -
Synonym(s) Bronchiolitis obliterans organizing pneumonia

Summary

Cryptogenic organizing pneumonia (COP) or bronchiolitis obliterans with organizing pneumonia (BOOP) is an inflammatory lung disease with distinctive clinical, radiological and pathological features. Men and women are affected equally and are usually aged between 50 and 60 years. The onset of symptoms is usually subacute with fever, nonproductive cough, malaise, anorexia and weight loss. Dyspnea is usually mild but is occasionally severe in some acute and life-threatening cases. No predisposing factors have been identified. In most cases, symptoms develop over a few weeks and the diagnosis of COP is made after 6 to 10 weeks. In COP, the organizing pneumonia is the most conspicuous pathological feature, examination of lung biopsy specimens shows intra-alveolar buds of granulated tissue consisting of fibroblasts, myofibroblasts, and loose connective tissue. The radiographic and computed tomography scan findings suggest the diagnosis when multiple patchy alveolar opacities with a peripheral and bilateral distribution are present. Video-assisted thoracoscopic lung biopsy is currently the preferred technique for diagnosing organizing pneumonia, because large lung specimens are necessary to make the diagnosis with confidence using other techniques. Transbronchial lung biopsy may show organizing pneumonia but does not allow the exclusion of associated disorders. Corticosteroids are the current standard treatment for COP. Response to this treatment is generally very good. *Authors: Prof. J-F. Cordier, Dr U. Costabel and Dr R. du Bois (October 2004)*.

Detailed information

Review article
Get Acrobat Reader
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.
Languages :