Cryptogenic organizing pneumonia
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
Age of Onset
Autosomal dominant ?A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease
Autosomal recessive ?Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype
X-linked dominant ?X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked recessive ?Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder
Mitochondrial or multigenic ?Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor ?Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Other Names (AKA)
COP; Idiopathic bronchiolitis obliterans organizing pneumonia; Idiopathic BOOP
Cryptogenic organizing pneumonia (COP) is a form of idiopathic interstitial pneumonia characterized by lung inflammation and scarring that obstructs the small airways and air sacs of the lungs (alveoli). Signs and symptoms may include flu-like symptoms such as cough, fever, malaise, fatigue and weight loss. COP often affects adults in midlife (40 to 60 years of age). The exact underlying cause of the condition is unknown (idiopathic). Treatment varies based on the severity of the condition but generally includes glucocorticoids.
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
|Medical Terms||Other Names||
|30%-79% of people have these symptoms|
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|Ground-glass opacification on pulmonary HRCT||0025179|
Elevated white blood count
High white blood count
Increased blood leukocyte number
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Increased blood neutrophil counts
|Restrictive ventilatory defect||
Stiff lung or chest wall causing decreased lung volume
|5%-29% of people have these symptoms|
|Bronchial breath sound||0031994|
Blue discoloration of the skin
Coughing up blood
|1%-4% of people have these symptoms|
Low blood oxygen level
[ more ]
Other forms of organizing pneumonia may result from infection (
Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.
Conditions with similar signs and symptoms from Orphanet
The main differential diagnosis is idiopathic chronic eosinophilic pneumonia. Secondary OP must also be excluded by elimination of its possible causes, including primary biliary cirrhosis, ulcerative colitis, Crohn disease, Sweet syndrome, sarcoidosis, Behçet disease, primary pulmonary lymphoma, drugs, infections, and all connective tissue diseases, especially rheumatoid arthritis and idiopathic inflammatory myopathies.
Visit the Orphanet disease page for more information.
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
- The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
- Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
- The Merck Manual for health care professionals provides information on Cryptogenic organizing pneumonia.
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Cryptogenic organizing pneumonia. Click on the link to view a sample search on this topic.
- Harold R. Collard. Cryptogenic Organizing Pneumonia. Merck Manual. 2015; https://www.merckmanuals.com/home/lung-and-airway-disorders/interstitial-lung-diseases/cryptogenic-organizing-pneumonia.
- Cordier JF. Cryptogenic organizing pneumonia. Clin Chest Med. December 2004; 25(4):727-738.
- Talmadge E King, Jr, MD. Cryptogenic organizing pneumonia. UpToDate. December 2014; Accessed 7/16/2015.
- Bronchiolitis Obliterans Organizing Pneumonia. NORD. 2013; https://rarediseases.org/rare-diseases/bronchiolitis-obliterans-organizing-pneumonia/.