The various manifestations, which are listed in the Table, may be coincident with, follow, or even precede the intestinal disease. Symptoms include cough, hoarseness, stridor, and dyspnea. Histology shows eroded epithelium and granulation tissue rich in plasma cells, lymphocytes, and PMNs. PFTs demonstrate airflow insufficiency. Cultures are usually negative.
Histology shows a lymphocytic bronchitis, focal squamous metaplasia, and luminal PMNs.
Inhaled steroids are beneficial in chronic bronchitis, but less so with CBS and bronchiectasis. Radiographs show diffuse small, irregular opacities. Histology resembles diffuse panbronchiolitis or sclerosing, constrictive bronchiolitis with little inflammation. Radiographs may have focal, unilateral, or diffuse opacities, often pleural-based with air bronchograms. PFTs show restriction.