Q: My 56-year-old husband, a picture of health, developed cryptogenic organizing pneumonia (COP ). It started off appearing to be a respiratory infection and long story short, it was diagnosed clinically by our family physician and then a pulmonologist as COP. It was officially diagnosed by tissue diagnosis with a VATS procedure. It is related to but not the same thing as BOOP. What information might you have or know about this condition? He is currently on 40 mg of prednisone for a month but was on more than that previously and is being weaned down.
In the midst of all this, this past week he developed DVT of the left leg which led to bilateral pulmonary emboli, which he is doing great from. Thank Heavens. He is now on Lovenox and Coumadin until his INR becomes therapeutic. But that’s another issue altogether. What I really want to know about is the COP. We are being told he should be over it by about December. One of the residents during the episode found studies about putting COP [patients on Biaxin as an adjunct to the prednisone and that it has helped some patients. So, he has now been started on that. This COP is something else!
Thanks and love your column. You help so many people.
A: Well, it appears you have had your hands full but are well-informed on your husband’s condition and are making the best of things. I’m not sure I can add a great deal to what you already know, but I will certainly attempt to do so.
Cryptogenic organizing pneumonia, previously known as bronchiolitis obliterans organizing pneumonia (BOOP) and idiopathic (cause unknown) bronchiolitis obliterans organizing pneumonia is an inflammatory lung disease that develops rapidly. The condition is a form of non-infectious pneumonia and is inflammation of the bronchioles and surrounding lung tissue. It is often a complication of an existing chronic inflammatory disease such as rheumatoid arthritis, or may be a side effect of amiodarone and certain other medications. Symptoms may include malaise, night sweats, fatigue, cough, fever, and weight loss. It is most prevalent in adults between the ages of 40 and 60. The cryptogenic form, secondary organizing pneumonia, is also found in correlation with connective tissue diseases, malignancy, a number of prescribed drugs, and other interstitial pneumonias.
The prevalence is unknown, yet a cumulative incidence of six or seven cases per 100,000 hospital admissions was found at a large teaching hospital in Canada; and a 20-year review of statistics nationwide for Iceland found the annual incidence was 1.1% per 100,000.
There are no predisposing factors identified as the cause of this disorder. This translates to any known cause for the pneumonia being ruled out prior to stating that organizing pneumonia is truly cryptogenic. Some organizing pneumonias may be the result of bacterial/viral or parasitic infection, toxic fumes, bronchial obstruction, drugs, or radiation therapy reaction for breast cancer. Organizing pneumonia may also occur in the context of specific disorders such as ulcerative colitis, post lung transplant and bone marrow grafting, or from certain connective tissue disorders. Those at an increased risk include individuals with scleroderma, lupus, and rheumatoid arthritis.
The diagnosis is often considered if there is no response to several antibiotic therapies and when blood and sputum cultures are negative for organisms. The symptoms of the condition are so commonly suggestive of an infection that most patients diagnosed with COP have been treated with at least one failed course of antibiotics prior to an accurate diagnosis being made. Approximately 75% of patients will have symptoms present for less than two months prior to seeking treatment. Physicians will not find specific abnormalities on routine lab work or through a complete examination, other than for the presence of rales (crackling sounds) when listening to the lungs with a stethoscope. However, pulmonary function tests will reveal the volume of oxygen in the blood to be uncommonly low at rest and becoming even lower with exercise. A chest X-ray will reveal white patches that are widespread; those patches appear to migrate from one area of the lung to another as the disease progresses and normal lung volumes.
Approximately two thirds of all patients so diagnosed recover with the assistance of corticosteroids with weaning over a 24 week period. The weaning period is extremely important because if the drug is reduced too quickly, the disease may return.
