Inflammatory bowel disease is recognized as being associated with a variety of bronchopulmonary disorders. This association was initially suggested in a 1976 report by Kraft1 that described 5 patients with ulcerative colitis and 2 patient with Crohn’s disease who had unexplained bronchial suppuration with and without recognized bronchiectasis. Subsequent reports have confirmed and expanded the association of respiratory tract involvement with inflammatory bowel disease.2 The reported manifestations have included asymptomatic lower tract inflammation,3 organizing pneumonia,4 nonspecific interstitial pneumonia,4 alveolitis, simple bronchitis, bronchiectasis,1 bronchiolitis,5 upper airway stenosis,6 and tracheobronchitis. Drug-induced lung disease has also been reported.5 Symptomatic bronchopulmonary disorders related to Crohn’s disease seem to be uncommon, and tracheobronchial involvement as an extraintestinal manifestation of Crohn’s disease is rare. We report an unusual case of severe tracheobronchitis in a man with Crohn’s colitis and review the English language literature detailing inflammatory involvement of the trachea and proximal bronchi in association with Crohn’s disease.
A 66-year-old nonsmoking man was previously healthy except for a limited bout of colitis in 1997. He was treated with mesalamine for 9 months with resolution of his symptoms. Symptoms recurred in December 2002 when he began experiencing abdominal cramping and diarrhea. A colonoscopy showed scattered ulcerations of the colonic mucosa extending from the ileocecal valve to the proximal sigmoid region. Biopsies exhibited active colitis with acute cryptitis, mucosal ulcerations, and a few granulomas consistent with Crohn’s disease. The patient was treated with sulfasalazine but was switched to mesalamine as a result of an allergic reaction. A repeat colonoscopy in May 2003 showed continued ulceration, unchanged from prior examination. Azathioprine was begun along with prednisone and a single infusion of infliximab. Gastrointestinal symptoms improved and he was tapered off prednisone and mesalamine.
In September 2003, he developed chest congestion and a productive cough with yellow–green and occasionally blood-tinged sputum. This was associated with fever, chills, and mild shortness of breath but no wheeze. His physical examination was unremarkable. A chest radiograph demonstrated thickening of the paratracheal soft tissues, which prompted chest computed tomography (CT). The CT demonstrated thickening of the trachea from the T3 level to the carina without mediastinal adenopathy or parenchymal abnormalities (Figs. 1 and 2). Sputum samples were negative for bacteria, fungi, and acid-fast bacilli. Pulmonary function testing showed moderate obstructive defect (FEV1 2.45 [66%], FVC 4.02 [83%], FEV1/FVC 61%) with normal carbon monoxide transfer.
A bronchoscopy demonstrated mucosal erythema without exudates and extensive nodularity that involved the entire trachea and extended to the subsegmental airways (Fig. 3). The segmental and subsegmental airways appeared edematous with lumenal narrowing. Tracheal mucosal biopsy showed papillary squamous epithelium with squamous metaplasia. The submucosa was chronically inflamed with associated inflammatory fibroepithelial polyps (Fig. 4). Rare giant cells were noted but there were no well-formed granulomas. Acid-fast and fungal studies were negative as were herpes and cytomegalovirus cultures. Inhaled budesonide, 400 μg twice a day, was started. After 3 weeks, the respiratory symptoms resolved. A repeat chest x-ray no longer suggested tracheal wall thickening.
We report a case of severe and unusual tracheobronchial inflammation associated with Crohn’s disease. A striking feature of this case was the tracheal wall thickening noted on both the chest radiograph and thoracic CT images. Subsequent endoscopy revealed an unusual diffuse nodularity of the mucosa with hyperemia and easy surface bleeding. The mucosa was reddish pink and had a “cobblestone” texture. There was no exudate, and bronchial secretions, although copious, were clear.
Table 1 lists data from the prior reports of tracheobronchitis and Crohn’s disease. In 3 of the reports,7–9 the mucosal surface displayed whitish nodules during bronchoscopic examination. These were described as granulations and were either diffuse and confluent or scattered. No white-coated mucosal lesions were seen in our case. Another report described such exuberant mucosal edema that the appearance was described as “pseudotumoral.”10 Our case did not manifest an appearance suggesting neoplasia. The nodules were small and diffuse, extending throughout the visible airways. The mucosal nodules seemed to be small polyps with intervening fissures, which could be linear ulcerations, hence, the cobblestone analogy. Mainstem bronchial stenosis has also been reported,6 but in our case, the main bronchi were not visibly narrowed. Only at the lobar and, especially, the segmental airways were the lumen appreciably narrowed.
Computed tomography of the chest was reported in 3 cases.6,9,10 No parenchymal abnormalities were described in these or in the case reported here. In 2 of the 3 CT studies, tracheal or main bronchial stenosis was observed.6,10 The sagittal diameter of the midtrachea in this case was 15 mm on the CT images and, assuming a normal diameter of 20 to 22 mm, indicates some lumenal narrowing. Tracheal wall thickening, similar to this case, was noted in 1 report.6 In the case reported here, the widening of the tracheal wall included stranding of tissue with increased attenuation that extended into the mediastinal fatty tissue. None of the reported plain chest radiographs revealed abnormalities. In this case, the plain radiograph, with thickening of the right lateral tracheal wall, served to prompt further investigation.
The histologic features in 3 of the reviewed cases mentioned granulomatous inflammation in the tracheobronchial mucosa.7–9 In 1 case, giant cells were observed.9 In all cases, there was a chronic inflammatory infiltrate in the submucosa that included lymphocytes and plasma cells. One case had a mixed infiltrate with neutrophilic leukocytes in the mucosa. Chronic inflammatory cells and fibroplasia were noted on biopsy material from our case but no granulomas were identified, although rare giant cells were observed (Fig. 4).
The current case demonstrates severe tracheobronchitis with airflow limitation in association with Crohn’s disease. We believe Crohn’s disease could have caused the tracheobronchitis. No infectious agent or alternative cause was identified. There are features in this case that are similar to that of other reports of tracheobronchial inflammation believed to represent an extraintestinal manifestation of Crohn’s disease. Antiinflammatory therapy (inhaled budesonide) was temporally associated with improved symptoms. The patient declined repeat investigations, but the posttreatment chest radiograph did not show tracheal wall thickening. Respiratory symptoms in patients with Crohn’s disease, especially when the latter is active, should be closely investigated. It is possible that tracheal and bronchopulmonary inflammation related to Crohn’s disease could be less rare than these few reports indicate.
1. Kraft SC, Earle RH, Rossler M, et al. Unexplained bronchopulmonary disease with inflammatory bowel disease
. Arch Intern Med
2. Camus P, Piard F, Ashcroft T, et al. The lung in inflammatory bowel disease
3. Bonniere P, Wallaert B, Cortot A, et al. Latent pulmonary involvement in Crohn’s disease
: biological, functional, bronchoalveolar lavage and scintigraphic studies. Gut
4. Casey MB, Tazelaar HD, Myers JL, et al. Noninfectious lung pathology in patients with Crohn’s disease
. Am J Surg Pathol
5. Storch I, Sachar D, Katz S. Pulmonary manifestations of inflammatory bowel disease
. Inflamm Bowel Dis
6. Kuzniar T, Sleiman C, Brugiere O, et al. Severe tracheobronchial stenosis in a patient with Crohn’s disease
. Eur Respir J
7. Lemann M, Messing B, D’Agay F, et al. Crohn’s disease
with respiratory tract involvement. Gut
8. Iwama T, Higuchi T, Imajo M, et al. Tracheo-bronchitis as a complication of Crohn’s disease
. Japanese Journal of Surgery
9. Lamblin C, Copin M-C, Billaut C, et al. Acute respiratory failure due to tracheobronchial involvement in Crohn’s disease
. Eur Respir J
10. Henry MT, Davidson LA, Cooke NJ. Tracheobronchial involvement with Crohn’s disease
. Eur J Gastroenterol Hepatol