Cryptogenic organizing pneumonia overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:
Overview
Cryptogenic organizing pneumonitis is a rapidly developing pneumonia-like illness characterised by lung inflammation and scarring that obstruct the small airways and air sacs of the lungs (alveoli). Its name is derived from the fact that it closely mimics pneumonia infections. (The term COP is used in Europe and BOOP in North America.)
Bronchiolitis obliterans organizing pneumonia is an inflammation of the bronchioles and surrounding tissue in the lungs. BOOP is often caused by a pre-existing chronic inflammatory disease like rheumatoid arthritis. In cases where no cause is found, the disease is called cryptogenic organizing pneumonia.
The clinical features and radiological imaging resemble infectious pneumonia. However, diagnosis is suspected after there is no response to multiple antibiotics, and blood and sputum cultures are negative for organisms.
Historical Perspective
Initial descriptions of organizing pneumonia can be traced back to 1877 Paris, in the lectures of J.M. Charcot. The first report of non-infectious organizing pneumonia without an identifiable underlying etiology was published in 1983. Soon after, the term "bronchiolitis obliterans organizing pneumonia (BOOP)" was introduced. This nomenclature was abandoned in 2002 by the American Thoracic Society/European Respiratory Society International Consensus Panel and the term cryptogenic organizing pneumonia was adopted.
Classification
There is no established system for the classification of cryptogenic organizing pneumonia.
Pathophysiology
Cryptogenic organizing pneumonia is an idiopathic diffuse interstitial lung disease that affects the distal bronchioles, respiratory bronchioles, alveolar ducts, and alveolar walls. The injury occurs within the alveolar wall. There is proliferation of granulation tissue which involves alveolar ducts and alveoli. There are 4 phases lead to the formation of mature fibrotic bud.
Causes
Differentiating Cryptogenic organizing pneumonia from Other Diseases
Epidemiology and Demographics
Risk Factors
Natural History, Complications, and Prognosis
Diagnosis
History and Symptoms
Physical Examination
Laboratory Findings=
Chest X Ray
CT
Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
The mainstay of the therapy is pharmacotherapy. Corticosteroids are used as first-line treatment for patients with the symptomatic and progressive disease. Treatment is planned according to the severity of the disease. For treatment of mild disease is to monitor if there is no worsening of symptoms or pulmonary function. If there is worsening of symptoms, macrolides are used in the treatment of mild disease.For persistent or gradually worsening disease, corticosteroids are used for treatment and for severe disease cytotoxic agents are added. Relapses are common with corticosteriods therapy, azathioprine is used.