Abstract

126 Crohn's disease, (CD) is a systemic disorder which has been reported to effect almost every organ system. We report an 11 year old boy who presented with respiratory symptoms, secondary to metastatic bronchopulmonary CD. He was admitted for treatment and investigation of dyspnea with exercise, and recurrent cough and fever which he had had over 10 months. Treatment with bronchodilators had not been helpful. Initial investigations revealed a mild anaemia with a hypochromic, microcytic blood film and hypoalbuminaemia, (albumin 24 g/l, total protein 58 g/l) leading to consideration of gastrointestinal pathology. The patient had had some mild abdominal pain and diarrhea for six weeks. On examination, (as well as significant respiratory signs), he had finger clubbing, peripheral edema, one small oral aphthous ulcer and two anal fissures. His chest X-ray showed patchy air space consolidation in both lower lobes. A CT scan of the chest revealed multiple peripheral nodules, suggestive of granulomatous disease, with no interstitial involvement. Pulmonary function testing demonstrated a reduced total lung capacity, decreased forced vital capacity and forced expiratory volume in one second, suggestive of restrictive lung disease. Bronchoscopy was normal, but the bronchoalveolar lavage identified frequent macrophages, suggestive of granulomatous inflammation. Histology of the lung biopsy showed grossly normal lung architecture with granulomatous inflammation in the nodules. Stains for mycobacteria and sarcoidosis were negative. Endoscopy revealed duodenitis as well as multiple aphthous ulcers throughout the colon. Histology showed granulomatous inflammation in gastric and colonic biopsies. Steroid therapy was recommended but was not started by the family because the symptoms were resolving. His CD has since relapsed with gastrointestinal symptoms and pancreatitis but no evidence of pulmonary involvement. The estimated incidence of pulmonary complications in inflammatory bowel disease is very small, with the majority of these occurring in patients with ulcerative colitis. This unusual presentation of CD reminds us that CD is a systemic disorder which frequently presents in childhood with predominantly extraintestinal manifestations.

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