Rare Rheumatology News

Disease Profile

Cryptogenic organizing pneumonia

Prevalence
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.

Unknown

Age of onset

Childhood

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ICD-10

J84.1

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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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

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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.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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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.

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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.

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Not applicable

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Other names (AKA)

COP; Idiopathic bronchiolitis obliterans organizing pneumonia; Idiopathic BOOP

Categories

Lung Diseases

Summary

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).[1] 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.[2][3][4]

Symptoms

Signs and symptoms of cryptogenic organizing pneumonia (COP) vary but may include:[3][4]

  • Persistent nonproductive cough
  • Difficult or labored breathing
  • Fever
  • Malaise
  • Weight loss
  • Hemoptysis (rare)

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
Learn More:
HPO ID
30%-79% of people have these symptoms
Anorexia
0002039
Crackles
0030830
Elevated erythrocyte sedimentation rate
High ESR
0003565
Fatigue
Tired
Tiredness

[ more ]

0012378
Fever
0001945
Ground-glass opacification on pulmonary HRCT
0025179
Leukocytosis
Elevated white blood count
High white blood count
Increased blood leukocyte number

[ more ]

0001974
Neutrophilia
Increased blood neutrophil counts
0011897
Nonproductive cough
Dry cough
0031246
Restrictive ventilatory defect
Stiff lung or chest wall causing decreased lung volume
0002091
5%-29% of people have these symptoms
Bronchial breath sound
0031994
Chest pain
0100749
Cyanosis
Blue discoloration of the skin
0000961
Elevated C-reactive protein level
0011227
Hemoptysis
Coughing up blood
0002105
Weight loss
0001824
Wheezing
0030828
1%-4% of people have these symptoms
Arthralgia
Joint pain
0002829
Hypoxemia
Low blood oxygen level
0012418
Night sweats
0030166
Pneumomediastinum
0025421
Pneumothorax
Collapsed lung
0002107
Respiratory distress
Breathing difficulties
Difficulty breathing

[ more ]

0002098

Cause

The underlying cause of cryptogenic organizing pneumonia (COP) is unknown (idiopathic). Organizing pneumonia is specifically diagnosed as COP when, among other characteristics, no definite cause for the organizing pneumonia is found. In other words, any known cause for the pneumonia must be ruled out before stating that a person is affected by COP.[2]

Other forms of organizing pneumonia may result from infection (bacteria, viruses, parasites, or fungi); drugs; or a reaction to radiation therapy for breast cancer. Organizing pneumonia can also be associated with specific disorders such as certain connective tissue disorders, blood malignancies (cancers), or ulcerative colitis.[2]

Diagnosis

A diagnosis of cryptogenic organizing pneumonia is often suspected based on the presence of characteristic signs and symptoms once other conditions that cause similar features have been excluded. This includes ruling out other known causes of organizing pneumonia. Additional testing such as a computed tomography (CT) scan or lung biopsy can confirm the diagnosis.[2][3]

Treatment

The treatment of cryptogenic organizing pneumonia (COP) generally depends on the severity of the condition. For example, people who are mildly affected may simply be monitored as some cases can improve on their own. Unfortunately, the majority of people with COP have persistent and/or progressive symptoms that will require therapy. In these cases, oral or intravenous glucocorticoids can be given which often result in rapid improvement of symptoms.[2][3]

Organizations

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.

Organizations Supporting this Disease

    Organizations Providing General Support

      Learn more

      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.

      Where to Start

        In-Depth Information

        • 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.

          References

          1. Harold R. Collard. Cryptogenic Organizing Pneumonia. Merck Manual. 2015; https://www.merckmanuals.com/home/lung-and-airway-disorders/interstitial-lung-diseases/cryptogenic-organizing-pneumonia.
          2. Cordier JF. Cryptogenic organizing pneumonia. Clin Chest Med. December 2004; 25(4):727-738.
          3. Talmadge E King, Jr, MD. Cryptogenic organizing pneumonia. UpToDate. December 2014; Accessed 7/16/2015.
          4. Bronchiolitis Obliterans Organizing Pneumonia. NORD. 2013; https://rarediseases.org/rare-diseases/bronchiolitis-obliterans-organizing-pneumonia/.

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