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 Idiopathic
BOOP Idiopathic BOOP Idiopathic BOOP is the most common form. It was described in 1985. The cause remains unknown. Men and women are equally affected. There is no relationship to smoking. A flu-like illness, fever and blood testing shows an increased sedimentation rate occur in 30 to 50 percent of patients. Cough is common, shortness of breath is mild. Wheezing and coughing blood are rare. 'Crackles' heard through the stethoscope occur in two-thirds of patients. The lung tests show a decrease in the "vital capacity' which is a test to determine the amount of air in the lungs. The 'diffusing capacity' is also decreased, this indicates that the inflammation in the lung is blocking oxygen transport into the blood. These test reductions are usually mild and may not be related to symptoms, but moderate and severe decreases are associated with shortness of breath. There is no obstruction of airflow except in smokers. The chest x-ray shows patchy densities in both lungs. Cavities in these 'patchy shadows' and fluid around the lungs are rare. The computerized lung scan (chest CT scan) shows similar patchy shadows. Sometimes, there are 'triangle' shadows in the films. These images represent inflammation in the shape of a triangle with the base of the triangle toward the chest wall and the tip toward the center of the chest. Laboratory studies often show a slight increase in the 'white cell count'. There often is an increase in the 'sedimentation rate'. This is a test that has been around for many years and a non-specific measure of an 'inflammatory' reaction. Because of the many lung diseases that can mimic BOOP and because of the different types and duration of treatment, lung tissue is usually needed to confirm the diagnosis of BOOP. Tissue can be obtained from a video assisted thoracoscopy (VAT) or from a small lung operation. The VAT procedure has become a common method for obtaining lung tissue. It is performed in a hospital setting and a small tube in the chest is needed for 24 to 48 hours. Prednisone continues to be the recommended medication for patients with pulmonary symptoms and progressive BOOP. The dosage and duration of prednisone are determined by several factors such as the person's weight, underlying disorders and severity of the illness. It may begin at 60 mgs per day for several days or for one to three months. It is then decreased to 40 mgs for one to three months, then 10 to 20 mgs daily for a total of one year. A shorter six month course may be sufficient in certain situations. Every other day dosage may be effective in some patients and may decrease the medication side effects. Insufficient amount or duration of steroid therapy will result in relapse, but fortunately the disorder will respond to the previous steroid response levels. Total and permanent recovery is seen in 65 to 80 percent of patients treated. The mortality (death) rate remains at about five percent. Erythromycin treatment for two to three months in 6 patients, corticosteroid inhaler for 8 months in one patient, and Cytoxan in one patient have been utilized successfully, but these are limited reports and remain 'experimental'. There is a rare form of BOOP referred to as accelerated or rapidly progressive BOOP. The duration of illness is generally a few days. It is a very serious form of the disease with severe symptoms and respiratory failure. Unfortunately, a high percentage of patients do not survive, but prompt initiation of corticosteroid therapy may be effective. |
