Types of 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.

Post infection BOOP

Post infection BOOP has been reported after adenovirus pneumonia, cytomegalovirus (CMV) pneumonia, influenza pneumonia, Legionella pneumonia, Chlamydia pneumonia, and malaria.

The underlying infection either resolves on its own as in some of the virus pneumonias or it is treated with antibiotics. As the pneumonia resolves, sometimes instead of disappearing, the pneumonia becomes 'organized' into 'inflammation'. This inflammation is often BOOP. Prednisone therapy is usually helpful and results in complete resolution.

Drug or medication BOOP

Several drugs have been associated with BOOP. These include long term antibiotics, an anti-irregular heart rate medication, and anti-cancer drugs.

The symptoms and response to therapy are similar to 'idiopathic' BOOP. Cough develops, then shortness of breath, and the chest x-ray shows patchy infiltrates. Corticosteroid therapy often results in complete resolution.

Focal nodular BOOP

Sometimes, the chest x-ray shows BOOP as a single rounded shadow. It has the appearance of a lung cancer. Because of this, the lesion is removed surgically. For the most part, these will not recur again.

In rare situations, this single lesion represents the beginning of typical 'idiopathic' BOOP. Prednisone therapy will then result in complete resolution.

Rheumatological or connective tissue BOOP

The rheumatological or connective tissue disorders include rheumatoid arthritis, lupus erythematosus and polymyositis. Although BOOP has been reported in virtually all connective tissue disorders, it is most commonly found in association with lupus erythematosus, polymyositis/dermatomyositis, and rheumatoid arthritis.

The clinical findings and radiographic features are similar to 'idiopathic' BOOP. When BOOP is associated with one of these disorders, it is often responsive to corticosteroid therapy, but the percentage of complete resolution is usually less than in the 'idiopathic' type.

Bone marrow transplantation BOOP

Bone marrow transplantation BOOP has been rarely reported. It may occur as a result of an infection with Mycoplasma, Cytomegalovirus or other infectious agents. Some patients have an excellent response to corticosteroid therapy. Too few reports are available to determine whether it is related to bone marrow transplant infections or represents a rejection process.

Lung transplantation BOOP

The characterization of BOOP in patients with lung transplantation continues to evolve. At first it was thought to be rare and related to infections, but reports have indicated that BOOP may occur in 10 to 25 percent of lung recipients.

The outcome in lung transplant recipients is less favorable than the 'idiopathic' form. The lung transplant BOOP may result from airway injury either from acute lung rejection or an ongoing infection.

AIDS or HIV infection BOOP<

In rare situations, BOOP has been reported to occur in patients with AIDS. It has been successfully treated with corticosteroid therapy.

Radiation therapy BOOP

In very rare situations, radiation therapy for treatment of lung cancer or breast cancer has resulted in BOOP in the location of the radiation treatment field. Corticosteroid treatment resulted in complete resolution for one patient.

Cancer and lymphoma-related BOOP

Occasionally, BOOP will be associated with cancers such as lung cancer, lymphoma, and leukemia. Surgical resection will cure the process in some patients. Corticosteroid therapy has variable results with complete resolution in some patients.

Seasonal BOOP

There was a report from England of eight women and four men aged 34 to 62 years who developed recurring pulmonary symptoms and liver abnormalities. Some of lung tissue specimens showed BOOP.

These features occurred every year in the last weeks of February and resolved by early May or earlier with corticosteroid therapy. Between relapses, patients were entirely symptom-free.The patients reported spontaneous remission of the first episode after a short illness. Eventually symptoms became more severe and corticosteroid therapy was often used which provided a rapid and complete resolution.The cause was not determined, although it was thought to be an inhaled agent present in the general environment from late February to early May, at least in England. It most likely is related to Ïallergic' response rather than an infection.

Inflammatory bowel disease BOOP

Inflammatory bowel disease is rare and includes ulcerative colitis and Crohn's disease.

BOOP has been reported in some of these patients. There was rapid and sustained clinical and roentgenographic improvement with corticosteroid treatment in most patients. There was complete resolution in two patients who were not treated because the BOOP was mild.

Tryptophan-related BOOP

L-Tryptophan is a popular supplement among health enthusiasts for use in sleep and pain disorders

There has been a report of a person who developed BOOP after taking 2.7 grams of L-tryptophan daily for 2.5 months.

Textile printing dye BOOP

There has been a report of 22 among 257 workers in eight textile airbrushing printing factories in Spain that developed a respiratory illness. The lung tissue of some of these workers showed BOOP.

It was suggested that spraying procedures that delivered a respirable aerosol of the textile printing dye caused the lung disease. The process was changed, and there have been no recurrences. Some of these workers responded to corticosteroid therapy, while others did not and some had residual symptoms.

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