ACR Codes: 68.2191
Bronchiolitis Obliterans Organizing Pneumonia (BOOP) is a disease entity characterized by granulation tissue plugging the terminal and respiratory bronchioles along with focal alveolar infiltrates. The exact pathogenesis is unknown, but it is believed to be a host response to airway injury. It is seen with a variety of diseases, including Legionella, viral, and mycoplasma infections, drag exposure, auto-immune disease, myelodysplastic syndrome, and inflammatory bowel disease. It can also be an idiopathic process. The clinical features of BOOP include cough, dyspnea, weight loss, and fever. It often mimics community acquired pneumonia. Symptoms usually evolve over two weeks to six months. Clinical findings include crackles on lung examination. Pulmonary function tests usually reveal a restrictive defect. On chest film, BOOP can present as patchy alveolar infiltrates (most common), nodular opacities, or linear opacities. The chest film can even be normal in 4-10% of patients. In BOOP related to connective tissue disease, linear opacities are seen more often than with other causes of BOOP. This was believed to be the case with this patient. CT scans reveal focal areas of consolidation, most often in the bases and peripherally, as was seen with this patient. The treatment for BOOP is corticosteroids. The standard therapy is 1mg/kg of prednisone for 1-3 months followed by a gradual taper for a total of one year of treatment. With treatment, approximately 65% of patients will have a complete remission. The remainder demonstrate some improvement or at least stabilization of their disease. For those who do improve, symptoms usually resolve in 2-3 days with radiographs normalizing in 2 to 4 weeks.
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Disease Topic 2332 
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