What are the other Names for this Condition? (Also known as/Synonyms)

• COP (Cryptogenic Organizing Pneumonia)
• Idiopathic BOOP
• Idiopathic Bronchiolitis Obliterans Organizing Pneumonia

What is Cryptogenic Organizing Pneumonia? (Definition/Background Information)

• 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

(Source: Cryptogenic Organizing Pneumonia; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Cryptogenic Organizing Pneumonia? (Age and Sex Distribution)

• Cryptogenic Organizing Pneumonia is a rare disorder
• The presentation of symptoms occur in middle-aged adults (between 40 and 60 years of age)
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Cryptogenic Organizing Pneumonia? (Predisposing Factors)

• Currently, no risk factors have been clearly identified for Cryptogenic Organizing Pneumonia

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Cryptogenic Organizing Pneumonia? (Etiology)

The underlying cause of Cryptogenic Organizing Pneumonia (COP) is unknown or 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
• 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
• Familial cases of Cryptogenic Organizing Pneumonia (COP) have not been reported in the medical literature, and presently, there is no evidence that some people may be genetically predisposed to developing COP

(Source: Cryptogenic Organizing Pneumonia; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

What are the Signs and Symptoms of Cryptogenic Organizing Pneumonia?

The signs and symptoms of Cryptogenic Organizing Pneumonia may include:

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

(Source: Cryptogenic Organizing Pneumonia; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How is Cryptogenic Organizing Pneumonia Diagnosed?

• 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

(Source: Cryptogenic Organizing Pneumonia; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Cryptogenic Organizing Pneumonia?

The complications of Cryptogenic Organizing Pneumonia may include:

• Respiratory distress
• Severe weight loss

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Cryptogenic Organizing Pneumonia Treated?

• 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

(Source: Cryptogenic Organizing Pneumonia; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How can Cryptogenic Organizing Pneumonia be Prevented?

• Currently, Cryptogenic Organizing Pneumonia may not be preventable, since the cause of the disorder is unknown
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Cryptogenic Organizing Pneumonia? (Outcomes/Resolutions)

• The long-term outlook (prognosis) for people with Cryptogenic Organizing Pneumonia (COP) is generally good
• Approximately two-thirds of affected people recover after treatment with glucocorticoids. Unfortunately, a third of affected people experience persistent symptoms and/or abnormalities on pulmonary function testing
• Rapidly fatal COP is uncommon

(Source: Cryptogenic Organizing Pneumonia; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Additional and Relevant Useful Information for Cryptogenic Organizing Pneumonia:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/