Respiratory involvement in patients with
inflammatory bowel disease (IBD) has been reported mainly since 1976. This form of involvement should clearly be separated from
interstitial lung disease due to
sulfasalazine or
mesalamine, although the distinction may be difficult in some cases. We report the data of an ongoing Registry containing 33 cases (23 cases receiving no
drug therapy) with
ulcerative colitis or, less often,
Crohn's disease, who developed varied bronchopulmonary problems. In several cases, the exact diagnosis and the relation of the bronchopulmonary disease to IBD had not been established for many years, thus delaying effective treatment with
steroids. In most cases (28/33), respiratory involvement followed the onset of IBD (8 of these 28 cases were postcolectomy), and in the remainder, respiratory manifestations predated the IBD. Patterns of involvement included: 1) Airway
inflammation, in the form of subglottic
stenosis,
chronic bronchitis, severe chronic bronchial
suppuration,
bronchiectasis, and chronic
bronchiolitis. In cases with large airway involvement, endoscopy showed exuberant inflammatory tissue in the airways and narrowing of tracheal and/or bronchial lumen. Histologically, airways were heavily infiltrated by a dense aggregate of inflammatory cells, and there were mucosal ulcerations. 2) Varied patterns of
interstitial lung disease, mainly
bronchiolitis obliterans with
organizing pneumonia, and pulmonary infiltrates and
eosinophilia. (3) Miscellaneous other forms of involvement including striking neutrophilic necrotic parenchymal nodules (corresponding histologically to sterile aggregates of neutrophils), and
serositis.
Steroids were very effective in the majority of cases. Inhaled
steroids were of durable benefit in patients with
chronic bronchitis, but less often so in those with chronic bronchial
suppuration,
bronchiectasis, or chronic
bronchiolitis.
Steroids administered orally led to marked improvement in patients with
interstitial lung disease and necrotic nodules, but lacked effectiveness in several patients with severe airway
inflammation or chronic
bronchiolitis. Intravenous
steroids were required in the initial management of life-threatening complications such as asphyxiating subglottic
stenosis or extensive
interstitial lung disease. Bronchial lavages with
methylprednisolone were effective in some patients with severe airway
inflammation. Patients with IBD can develop varied inflammatory complications in the lung, and a sizable fraction of these complications is
steroid-sensitive.