Cryptogenic Organizing Pneumonitis
Cryptogenic organizing pneumonitis (also called idiopathic bronchiolitis obliterans with organizing pneumonia) is a rapidly developing pneumonia-like illness characterized by lung inflammation and scarring that obstruct the small airways and air sacs of the lungs (alveoli).
The cause of cryptogenic organizing pneumonitis is unknown. The disease usually begins between the ages of 40 and 60 and affects men and women equally.
Almost 75% of people have symptoms for less than two months before seeking medical attention. A flu-like illness, with a cough, fever, a feeling of illness (malaise), fatigue, and weight loss heralds the onset in about 40% of people.
Doctors do not find any specific abnormalities on routine laboratory tests or on a physical examination, except for the frequent presence of crackling sounds (called Velcro crackles) when the doctor listens with a stethoscope. Pulmonary function tests usually show that the amount of air the lungs can hold is below normal. The amount of oxygen in the blood is often low at rest and is even lower with exercise.
The chest x-ray is distinctive with features that appear similar to an extensive pneumonia, with both lungs showing widespread white patches. The white patches may seem to migrate from one area of the lung to another as the disease persists or progresses. Computed tomography (CT) may be used to confirm the diagnosis. Often, the findings are typical enough to allow the doctor to make a diagnosis without ordering additional tests.
To confirm the diagnosis, a doctor may perform a lung biopsy using a bronchoscope (see Section 4, Chapter 39). Many times, a larger specimen is needed and must be removed surgically. About two thirds of people recover with corticosteroid therapy.
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