Show Summary Details
Page of

Bronchiolitis obliterans and cryptogenic organizing pneumonia 

Bronchiolitis obliterans and cryptogenic organizing pneumonia

Chapter:
Bronchiolitis obliterans and cryptogenic organizing pneumonia
Author(s):

A.U. Wells

and Nicholas K. Harrison

DOI:
10.1093/med/9780199204854.003.181103

A relevant case history from Oxford Case Histories in Respiratory Medicine has been added to this chapter.

Page of

PRINTED FROM OXFORD MEDICINE ONLINE (www.oxfordmedicine.com). © Oxford University Press, 2015. All Rights Reserved. Under the terms of the licence agreement, an individual user may print out a PDF of a single chapter of a title in Oxford Medicine Online for personal use (for details see Privacy Policy).

date: 24 March 2017

The nomenclature of the bronchiolitides is complicated by the interchangeable use of pathological and clinical descriptions and a diversity of classification systems. The four primary histological patterns are (1) organizing pneumonia (also termed proliferative bronchiolitis and bronchiolitis obliterans organizing pneumonia); (2) bronchiolitis obliterans (also termed obliterative bronchiolitis and constrictive bronchiolitis); (3) follicular bronchiolitis; and (4) diffuse panbronchiolitis.

Organizing pneumonia—the most characteristic abnormality is a filling of alveoli with granulation tissue and buds of loose collagen and connective tissue matrix cells with a uniform appearance. Presentation is typically subacute with nonproductive or minimally productive cough, insidious dyspnoea, and systemic symptoms including malaise, fever or chills, weight loss, and myalgia. Clinical signs are nonspecific. The chest radiograph most commonly shows patchy bilateral peripheral consolidation, which is often basal, and serial radiographs often show migration of infiltrates. High-resolution CT most often shows focal subpleural consolidation, with or without air bronchograms. Corticosteroid therapy is usually effective, with other immunosuppressive agents given to fulminant cases or those that do not respond. Prognosis is usually good, with overall mortality less than 5%.

Bronchiolitis obliterans—results from progressive obliteration of the terminal bronchioles with connective tissue matrix, which is cryptogenic in most cases. The usual presentation is with progressive breathlessness and the most characteristic physical finding is of inspiratory ‘squawks’, which are reliably indicative of small airway disease. High-resolution CT is often diagnostic, revealing focal areas of decreased attenuation representing regional gas-trapping and associated hypoperfusion, termed ‘mosaic attenuation’ or ‘mosaic perfusion’. The disease is not responsive to treatment, but in cases of diagnostic uncertainty it is usual to institute a trial of corticosteroids.

Follicular bronchiolitis—results from polyclonal hyperplasia of lymphoid follicles with formation of germinal centres within the bronchiolar walls. Patients usually present with progressive breathlessness, cough, and symptoms of recurrent respiratory infection. high-resolution CT invariably reveals centrilobular nodules less than 3 mm in diameter. Prognosis is generally good.

Diffuse panbronchiolitis—is characterized by bronchiolocentric inflammation, lymphoid hyperplasia, and an accumulation of interstitial foam cells in the lungs. Patients (most typically Japanese) present with subacute symptoms of cough productive of purulent sputum, dyspnoea, and sometimes weight loss. Survival has been transformed by the use of long-term, low-dose erythromycin therapy.

Access to the complete content on Oxford Medicine Online requires a subscription or purchase. Public users are able to search the site and view the abstracts for each book and chapter without a subscription.

Please subscribe or login to access full text content.

If you have purchased a print title that contains an access token, please see the token for information about how to register your code.

For questions on access or troubleshooting, please check our FAQs, and if you can''t find the answer there, please contact us.