Disease: Diffuse Panbronchiolitis
Clinical Information:
This is a 68 y. o. male diagnosed with "asthma" as a child.
He describes an episode of pneumonia at age 7 and throughout
young adulthood he had a chronic cough. He has a 72 pack
year history of smoking. Pulmonary function studies and
exercise physiology showed hypercapnia and hypoxemia. FVC,
FEV1 and DLCO were decreased. Rheumatologic markers and
viral/fungal antigen titers were negative.
Imaging:
The CXR shows increased lung volumes, marked
bronchial wall thickening and small nodule formation.
HRCT confirms these findings plus demonstrates dfiffuse peripheral
bronchietasis, mucus plugs and emphysema.
Discussion:
Diffuse panbronchiolitis is a disease that has been primarily
described in Asian males. Symptoms include productive cough
and dyspnea and in some patients sinusitis.
Histopathologically, inflammation of terminal and respiratory
bronchiole walls with lymphocytes and plasma cells leads to
bronchitectasis. Peribronchial inflammation and fibrosis are
manifested by centrilobular nodules. The disease has rarely
been described in Caucasians and in patients with ulcerative
colitis.
References:
Yousem, S. in Saldano,M. ed. Pathology of Pulmonary Disease
Lippincott, Philadelphia. 1994. pp. 318-320.
Case contributed by Valerie Hale, MD.
Debra Dyer, MD (Deb.Dyer@UCHSC.edu)
