Inflammatory bowel diseases are well known for producing a wide variety of extraintestinal manifestations. Pulmonary involvement is an uncommon extraintestinal manifestation, and can affect any part of the bronchopulmonary system. We present here the case of an adolescent female with Crohn’s colitis with bronchial involvement.
A 9-year old female presented to her physician with fatigue and anemia. Following a thorough workup, ileocolonoscopy and esophagogastroduodenoscopy at that time revealed colitis only of the left side and transverse portions of her colon. Her symptoms improved with steroid therapy, but she would relapse once the steroids were stopped. Symptoms included up to 10 bowel movements a day, abdominal cramping, urgency, and weight loss. Additional medical therapy included mesalamine, methotrexate, Pentasa, 6-mercaptopurine, and granulocyte colony stimulating factor. During treatment for her intestinal symptoms, she developed a persistent cough. Computed tomography (CT) scan of the chest demonstrated reactive pulmonary nodules. Infectious workup of the nodules was unremarkable, and it was thought that the nodules represented reactivity to the inflammatory bowel disease. Subsequent gastrointestinal biopsies revealed pancolitis, and the diagnosis of Crohn’s disease was suggested. She began treatment with infliximab, and an attempt at weaning her off steroids was met with an exacerbation of her gastrointestinal (GI) symptomatology. All throughout, she continued to have a slightly productive cough that waxed and waned with her GI symptoms. Repeat CT scan of the chest demonstrated mild pericarinal and hilar adenopathy. Because of the new adenopathy and long-standing nearly-chronic immunosuppression, it was decided to once again rule out infection. Flexible bronchoscopy revealed mild laryngomalacia with inspiratory prolapse of the left arytenoid into the supraglottic fossa. The vocal cords were normal. Starting at the midtrachea and extending to the distal bronchial tree bilaterally, the mucosa was significantly edematous, erythematous, friable, and diffusely nodular. Several of the mucosal nodules measured up to 3 mm X 3 mm. These nodules partially obstructed segmental bronchi particularly bronchi to the medial basal segment of the right lower lobe, lateral basal segment of the left lower lobe, and one of the subsegmental bronchi to the superior segment bronchus right lower lobe. Diagnostic broncho-alveolar lavage from the right middle lobe segments were nondiagnostic and did not reveal infection. Multiple large biopsies were taken from the mucosal nodularities in the right bronchial tree. The biopsies showed ulceration with focal suppurative inflammation and dense tissue eosinophilic infiltrate. All stains for organisms, including acid-fast, were negative.The patient continued undergoing medical treatment for her inflammatory bowel symptoms. Subsequent attempts to reduce the steroid doses were met with further exacerbation of both her gastrointestinal and bronchopulmonary symptoms.
Both chronic ulcerative colitis and Crohn’s disease are known to produce a variety of extraintestinal manifestations. Although pulmonary involvement is an uncommon manifestation of these diseases, 36% to 68% of patients with Crohn’s disease have abnormal pulmonary function. Pulmonary abnormalities can present simultaneously with active bowel disease, but can also present years after the onset of the bowel disease. The pathogenesis of pulmonary disease in inflammatory bowel disease is not known, but a common systemic mechanism affecting both the bronchial and gastrointestinal epithelium is likely responsible. It is essential to rule out infection as the main etiology for pulmonary disease in patients who are immunosuppressed. It is also possible that the drug therapy administered to treat the inflammatory bowel disease symptoms may also contribute to pulmonary pathology.
In patients with inflammatory bowel disease, deterioration of pulmonary function and an increase in pulmonary symptoms can parallel underlying disease activity. Medications used to control gastrointestinal symptoms of irritable bowel disease generally also alleviate the extraintestinal bronchopulmonary symptoms.
Jess Thompson, No Financial Disclosure Information; No Product/Research Disclosure Information