What is Cryptogenic Organizing Pneumonia (COP)?

Cryptogenic organizing pneumonia (COP), previously known as bronchiolitis obliterans with organizing pneumonia (BOOP), is a rare lung condition in which inflammation and scarring obstruct the bronchioles (smallest airways), the walls of the bronchioles and the tiny, grape-like alveoli (air sacs) deep within the lungs.^[1] COP is a form of idiopathic interstitial pneumonia in which the cause is unknown.

Signs and Symptoms of COP

Symptoms of COP usually begin gradually, over a period of a few weeks to a few months. The most common symptoms include:^[2]

• Persistent, non-productive cough, sometimes lasting 2 to 4 months
• Shortness of breath
• Low-grade fever
• Malaise (general feeling of being unwell)
• Loss of appetite and weight loss

Making a COP Diagnosis

A diagnosis of cryptogenic organizing pneumonia (COP) starts with a thorough physical examination and your doctor asking you many questions about your health and medical history. Your doctor may also order special, diagnostic tests that may include:²

• Chest X-ray
• Lab tests
• Pulmonary function tests
• Lung biopsy

Treatment of COP

COP sometimes resolves on its own, but most of the time, treatment, which may include the following, is required:²

• Corticosteroids – the gold standard, treatment of choice for COP is with a corticosteroid such as prednisone. Prednisone reduces the inflammation associated with the disease.
• Cytotoxic drugs – if corticosteroids are ineffective, a cytotoxic drug such as cyclophosphamide may be used. Cyclophosphamide is a type of antineoplastic drug that is used to treat cancer.
• Flu and pneumonia vaccine – your doctor may recommend you get a flu and pneumonia vaccine to help keep you healthier

Note: because COP is not an infection, it does not respond to treatment with antibiotics.²

Living with COP

Most people with cryptogenic organizing pneumonia (COP) require weeks or months of treatment with a corticosteroid such as prednisone. Oftentimes after taking this drug, improvement is seen within a few days or weeks. One down side of taking a drug like prednisone is that, as your dosage of the drug is reduced, which is necessary before completely stopping a corticosteroid, COP can reoccur. If this happens, another course of treatment is warranted.²

Cryptogenic Organizing Pneumonia Prognosis

The prognosis (long-term outlook) for people living with COP is generally very good. Approximately two-thirds of those affected will recover after treatment with corticosteroids. Regrettably, one-third with COP will experience recurrent symptoms and/or pulmonary function test abnormalities.²

When to See Your Doctor

It’s important that, if you develop a persistent, non-productive cough or shortness of breath that doesn’t go away, you see your doctor right away.  The quicker you begin treatment for COP, the quicker it will go away, especially if you are very young, older than 65 or have other chronic health problems such as COPD.²

For more information about cryptogenic organizing pneumonia (COP), contact your primary care provider. You can also visit the American Lung Association’s Living with Lung Disease Support Community to be connected to others facing this disease. Call their Lung Helpline at 1-800-LUNGUSA for more information or to be connected to support.

_{[1] Moua, Teng MD. Cryptogenic organizing pneumonia. Mayo Clinic. September 23, 2015.}

_{[2] Carbone, Roberto MD, FCCP. CHEST Foundation. Last reviewed January, 2018.}