Two cases of diffuse panbronchiolitis in a family were reported. They had chronic cough productive of a large amount of sputum for more than 10 years. Their clinical pictures, chest X-ray and bronchography demonstrated diffuse panbronchiolitis. Chronic nasal sinusitis was identified. Their tuberculin skin tests were negative and the titers of the cold hemagglutinin test were moderately elevated (X 256). Their serum complement levels (C3) were decreased.
The first case was a 73-year old mother, who was also diagnosed as having chronic thyroiditis by thyroid scintigram. Her serum T4 level was only 1.6μg/dl, TSH 113.8μU/ml, anti-thyroid antibody 25600x and anti-microsome antibody 6400x.
The second case was her 49-year old daughter, who was also diagnosed as having selective IgA deficiency. Her serum IgA was below 10mg/dl with increased serum levels of IgG and IgM. IgA were not detected in her saliva and sputum though secretory component was detected in saliva, 12μg/dl.
Many other members of the family had chronic nasal sinusitis, chronic thyroiditis and rheumatoid arthritis, and had auto-antibosies and abnormal levels in serum immunogolbulins. These two cases suggest that immunologic deficiency might play an important role in the pathogenesis and the mechanisma of the progression of diffuse panbronchiolitis.
