Am. J. Respir. Crit. Care Med., Vol 154, No. 2, Aug 1996, 497-503.
Diffuse panbronchiolitis in the United States
JE Fitzgerald, TE King Jr, DA Lynch, RM Tuder and MI Schwarz
Department of Medicine, University of Colorado Health Sciences Center, Denver 80262, USA.
Diffuse panbronchiolitis (DPB), an important cause of progressive
obstructive lung disease in the Far East, represents a distinctive
sinobronchial syndrome with typical radiologic and histologic features. We
have identified DPB in five citizens of the United States, three with
histologic confirmation, who have never traveled to the Far East. There
were four men and one woman, whose ages ranged from 46 to 75 yr at the time
of diagnosis. All had a prior history of chronic sinusitis and presented
with cough, dyspnea, and sputum production. Three were never smokers and
two were current smokers. Pulmonary function testing revealed severe
airflow limitation (the FEV1 ranging from 22% to 56% of predicted), and
overdistention. All patients had high-resolution computed tomographic
(HRCT) scans indicating centrilobular nodules with adjoining thickened and
dilated bronchioles. In the three patients in whom open lung biopsy was
performed, there was bronchiolocentric infiltration of lymphocytes, plasma
cells, and foamy macrophages. Three patients remain alive and are being
treated with chronic macrolide therapy. The clinical, radiographic, and
histologic features of these patients closely resemble those described in
Japanese patients. DPB must be considered in the differential diagnosis of
sinopulmonary syndromes, bronchiolitis, and cryptic cases of obstructive
lung disease among United States citizens, since therapy now offers an
improved prognosis.
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Copyright © 1996 American Thoracic Society
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