Emergency: requires immediate attention
Cryptogenic organizing pneumonia
Alerts and Notices
Synopsis

Formerly known as the idiopathic form of bronchiolitis obliterans organizing pneumonia (BOOP), cryptogenic organizing pneumonia (COP) is a diffuse interstitial lung disease affecting distal and respiratory bronchioles and alveolar ducts and walls. It usually presents in patients aged 50-60 years, with men and women equally affected, and is frequently heralded by a flu-like illness.
Patients typically present with relatively short duration of symptoms (weeks to months) including a persistent nonproductive cough, dyspnea, fever, malaise, weight loss, and occasionally other influenza-like symptoms (nasal congestion, headache, chills and sweats, sore throat, myalgia). Hemoptysis is uncommon. Physical examination reveals inspiratory crackles in a majority of patients, and radiographic findings include peripheral, patchy bilateral opacities.
History of stigmata of extrapulmonary connective tissue disease and/or medication exposure should be sought to differentiate COP from other idiopathic interstitial pneumonitides.
Patients typically present with relatively short duration of symptoms (weeks to months) including a persistent nonproductive cough, dyspnea, fever, malaise, weight loss, and occasionally other influenza-like symptoms (nasal congestion, headache, chills and sweats, sore throat, myalgia). Hemoptysis is uncommon. Physical examination reveals inspiratory crackles in a majority of patients, and radiographic findings include peripheral, patchy bilateral opacities.
History of stigmata of extrapulmonary connective tissue disease and/or medication exposure should be sought to differentiate COP from other idiopathic interstitial pneumonitides.
Codes
ICD10CM:
J84.116 – Cryptogenic organizing pneumonia
SNOMEDCT:
719218000 – Cryptogenic organizing pneumonia
J84.116 – Cryptogenic organizing pneumonia
SNOMEDCT:
719218000 – Cryptogenic organizing pneumonia
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Hypersensitivity pneumonitis
- Infectious pneumonia, including bacterial (eg, pneumococcal pneumonia), viral, or fungal
- Chronic eosinophilic pneumonia
- Pulmonary lymphoma
- Diffuse alveolar damage (acute respiratory distress syndrome)
- Drug-induced pneumonitis
- Diffuse alveolar hemorrhage
- Pulmonary manifestation of connective tissue disease (rheumatoid arthritis, polymyositis / dermatomyositis, systemic sclerosis, Sjögren syndrome, systemic lupus erythematosus, ankylosing spondylitis)
- Pulmonary manifestation of inflammatory bowel disease (ulcerative colitis or Crohn disease)
- Granulomatosis with polyangiitis
- Aspiration pneumonitis / aspiration pneumonia
- Inhalation injury (eg, smoke inhalation)
- Irradiation injury (radiation pneumonitis)
- Desquamative interstitial pneumonia
- Nonspecific interstitial pneumonia
- Lymphoid interstitial pneumonia
- Usual interstitial pneumonia (idiopathic pulmonary fibrosis)
Best Tests
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Management Pearls
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
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References
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Last Reviewed:05/17/2022
Last Updated:06/19/2022
Last Updated:06/19/2022
Emergency: requires immediate attention
Cryptogenic organizing pneumonia
