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