Cryptogenic Organizing Pneumonia

What is cryptogenic organizing pneumonia?

Cryptogenic organizing pneumonia (COP) is a rare lung condition that affects the air sacs (alveoli) and small airways (bronchioles) of your lungs. COP causes lung inflammation and can cause lung scarring.

Cryptogenic organizing pneumonia often starts with a flu-like illness, but it’s not a true infection. COP can progress into a worsening cough with shortness of breath while moving (exertion).

Healthcare providers usually prescribe corticosteroids to treat COP after making sure there isn’t an infection. Most people make a complete recovery, but this can take time. Sometimes symptoms come back despite treatment.

Who might get cryptogenic organizing pneumonia?

Cryptogenic organizing pneumonia can affect anyone no matter their gender, ethnicity or race. COP often affects people aged 40 to 60 years old. Most people with COP don’t smoke.

Your risk of getting cryptogenic organizing pneumonia may increase if you have an inflammatory disease, such as:

• Lupus.
• Rheumatoid arthritis.
• Scleroderma.
• Multiple sclerosis.
• Inflammatory bowel disease (Crohn’s disease or ulcerative colitis).

How common is it?

COP affects 1 to 3 out of 100,000 people admitted to the hospital in the United States.

What causes cryptogenic organizing pneumonia?

Cryptogenic organizing pneumonia is a kind of idiopathic diffuse interstitial lung disease. The words “cryptogenic” and “idiopathic” mean that health providers don’t know the exact cause. Usually, the healthcare provider will rule out an infection (from bacteria, a virus, or fungi) and prior medications as causes before diagnosing COP.

Conditions associated with cryptogenic organizing pneumonia include:

• Cancer of the lymphatic system (lymphoma).
• Lung abscesses.
• Lung cancer.
• Hematological (white blood cell or red blood cell) cancers.

What are the symptoms of cryptogenic organizing pneumonia?

Fifty percent of people with COP have flu-like symptoms. These symptoms usually include:

• Chills.
• Dry cough (nonproductive cough).
• Fatigue (malaise).
• Loss of appetite.
• Fever.
• Unexplained weight loss.
• Night sweats.

About 75% of people then develop symptoms that include:

• Chronic cough.
• Shortness of breath (dyspnea) with exertion.

More rarely, symptoms may include:

• Chest pain.
• Collapsed lung (pneumothorax).
• Coughing up blood.
• Joint pain.
• Low oxygen levels in your blood (hypoxemia).

How is cryptogenic organizing pneumonia diagnosed?

Your healthcare provider will first ask about your symptoms and do a physical exam. They will need to rule out other conditions with similar symptoms, such as:

• Secondary organizing pneumonia (due to infection, medications or an autoimmune issue).
• Bacteria, fungal or viral pneumonia.
• Chronic eosinophilic pneumonia (eosinophilia in your lungs).
• Hypersensitivity pneumonitis.
• Chronic lung diseases.
• Primary pulmonary lymphoma.

During your physical exam, your provider will listen to your lungs with a stethoscope. They may hear crackling sounds and squeaking sounds when you inhale.

How does cryptogenic organizing pneumonia differ from organizing pneumonia?

Secondary organizing pneumonia (OP) has similar symptoms to cryptogenic organizing pneumonia. But unlike COP, infections, medications, cancers and autoimmune issues can cause secondary OP. Providers have also linked secondary OP to:

• Cancer that impacts blood-forming tissue (hematologic malignancy).
• Drug exposures.
• Radiation exposure to your lungs.

Your provider will want to rule out secondary OP before they start treatment. Treatment for secondary OP is different than treatment for COP. People with secondary OP also tend to have worse outcomes.

What tests help to diagnose cryptogenic organizing pneumonia?

If your provider suspects COP, they may suggest tests, including:

• Chest X-ray: Uses beams of radiation to create an image of your lungs. This image can help your provider diagnose COP.
• High-resolution CT (computed tomography) scan (HRCT): Takes multiple x-rays of your chest to visualize the lungs and inflammation caused by COP.
• Bronchoscopy with bronchoalveolar lavage (BAL): Your provider will put sterile saline solution through a thin tube with a light and camera on the end (bronchoscope). They collect a fluid sample from your lungs. Your provider may find increased white blood cells if you have COP, but more importantly, this helps rule out an infection.
• Lung biopsy: During a transbronchial lung biopsy, your provider removes a sample of lung tissue. This biopsy helps them rule out other conditions. Sometimes your provider may need to use a surgical lung biopsy to test more lung tissue.
• Pulmonary function testing: Your provider uses pulmonary function testing to see how well your lungs work. If you have COP, you can usually hold less air in your lungs than normal. You may have low oxygen in your blood when you rest and lower oxygen during exercise.

How is cryptogenic organizing pneumonia treated?

Providers usually treat cryptogenic organizing pneumonia with corticosteroids, such as prednisone. You will usually take this medication for 3 to 6 months with higher doses at first, then slowly come down. Some symptoms can come back if the corticosteroids are lowered too quickly. This is commonly seen when the prednisone is lowered past 20 mg a day. Mild cases of COP may go away without medications.

If COP symptoms continue or you are unable to tolerate the corticosteroids due to side effects, your provider may suggest a steroid-sparing agent like azathioprine or cytotoxic medications, such as cyclophosphamide, temporarily.

How can I reduce my risk of cryptogenic organizing pneumonia?

You can’t reduce your risk of COP. But you can look for the signs so you can get prompt monitoring and treatment, if needed.

What are the complications of cryptogenic organizing pneumonia?

Without treatment, COP can cause serious lung damage with lung scarring.

What is the outlook if I have cryptogenic organizing pneumonia?

Most people with cryptogenic organizing pneumonia recover quickly. About 50% of people with mild cases of COP recover without treatment. Up to 80% of people treated with corticosteroids completely recover from COP.

But COP symptoms may return. Treatment with corticosteroids usually helps to improve symptoms if they happen again. People who have recovered from COP usually live long, full lives.

How do I take care of myself with cryptogenic organizing pneumonia?

See your healthcare provider as soon as possible if you have cryptogenic organizing pneumonia symptoms. Prompt treatment can help prevent future episodes of COP.

If your provider prescribes corticosteroids to treat COP, let them know about any side effects. They can adjust your medication as needed.

A note from Cleveland Clinic

Cryptogenic organizing pneumonia (COP) is a rare lung condition that causes lung inflammation and scarring. Common COP symptoms include cough and shortness of breath. Healthcare providers usually treat COP with corticosteroids, though mild COP may go away on its own. Cryptogenic organizing pneumonia may come back again. But people with COP usually recover fully and can lead long lives.