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An unusual complication of ulcerative colitis

Q2: What is the likely diagnosis and what further investigations should be performed to confirm this?
The most likely cause of predominantly mid and upper lobe bronchiectasis is allergic bronchopulmonary aspergillosis. This results from a hypersensitivity reaction to inhaled fungal spores with mucus plug formation and bronchiectasis.

To make this diagnosis there must be evidence of sensitisation to aspergillus by positive skin allergen testing or serum precipitins, along with either upper lobe bronchiectasis (confirmed by bronchoscopy), raised total IgE or positive sputum cultures.¹ All of these results were found in our patient and confirmed the diagnosis.

Q3: What treatment should the patient be given?
The patient should be given oral steroids. This patient responded with dramatic symptomatic improvement, weight gain, and radiographic resolution.

Q4: What are the pulmonary complications of ulcerative colitis?
Various pulmonary disorders have been reported as extraintestinal manifestations of ulcerative colitis. These include pulmonary vasculitis, obliterative bronchiolitis, and a well documented association with chronic bronchial suppuration and bronchiectasis.^2–4 These tend to progress independently of colonic disease activity and onset years after colectomy is not uncommon.⁵

Pulmonary complications associated with sulphasalazine therapy include hypersensitivity pneumonitis, fibrosing alveolitis, and bronchiolitis obliterans.^6–8 These conditions develop within months of starting treatment. There have been no previous reports of allergic bronchopulmonary aspergillosis in association with inflammatory bowel disease.

A common physiological process linking ulcerative colitis and allergic bronchopulmonary aspergillosis is difficult to substantiate. However this does not preclude an association, as it is similarly difficult to find evidence explaining the association with other pulmonary disorders.

Interestingly our patient had a positive p-ANCA titre and a reported series suggests that patients with ulcerative colitis and bronchiectasis have an increased frequency of autoantibodies.⁴ It was proposed that a T-lymphocyte associated defect may lead to development of antibodies to a range of tissue antigens and destruction of various mucosal surfaces.

Allergic bronchopulmonary aspergillosis may be a rare association of ulcerative colitis and should be considered as a potential diagnosis in patients who fulfill the diagnostic criteria.

Final diagnosis
Allergic bronchopulmonary aspergillosis in association with ulcerative colitis.

References

1. Pasteur M, Helliwell S, Houghton S, et al. An investgation into causative factors in patients with bronchiectasis. Am J Respir Crit Care Med 2000;162:1277–84.[Abstract/Free Full Text]
2. Isenberg J, Goldstein H, Rosen V. Pulmonary vasculitis—an uncommon complication of ulcerative colitis. N Engl J Med 1968;279:1376–7.
3. Swinburn C, Jackson G, Corris P. Bronchiolitis obliterans organising pneumonia in a patient with ulcerative colitis. Thorax 1988;43:735–6.[Abstract/Free Full Text]
4. Butland R, Cole P, Citron K, et al. Chronic bronchial suppuration and inflammatory bowel disease. Q J Med 1981;50:63–75.[Abstract/Free Full Text]
5. Wilcox P, Miller R, Miller G, et al. Airway involvement in ulcerative colitis. Chest 1987;92:18–21.[Abstract/Free Full Text]
6. Leino R, Lippo K, Ekfors T. Sulphasalasine induced reversible hypersensitivity pneumonitis and fatal fibrosing alveolitis. J Intern Med 1991;229:553–6.[Medline]
7. Thomas P, Seaton A, Edwards J. Respiratory disease due to sulphasalasine. Clin Allergy 1974;4:41–7.[Medline]
8. Williams T, Eidus L, Thomas P. Fibrosing alveolitis, bronchiolitis obliterans and sulphasalasine therapy. Chest 1982;81:766–8.[Abstract]

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