Tracheobronchitis With Stridor in a Patient With Ulcerative Colitis

Abstract

Bronchopulmonary involvement is a rare but well documented extra-intestinal manifestation of inflammatory bowel disease (IBD). IBD related pulmonary disease can range from subglottic-glottic stenosis to tracheobronchitis to interstitial lung disease and is often misdiagnosed on initial presentation. Symptoms often do not correlate with IBD activity and can even manifest in those with undiagnosed IBD. We present a case of Ulcerative Colitis (UC) related tracheobronchitis in a patient who presented with stridor to emphasize the importance of maintaining a clinical suspicion for pulmonary manifestations of IBD. A 23 year-old woman with UC, who had recently been treated for pneumonia, returned to the emergency department due to worsening respiratory distress and stridor. Past medical history was significant only for UC for which she had self-discontinued mesalamine as she was symptom free. Tracheobronchitis was evident on computed tomography (CT) of the neck and thorax which showed diffuse circumferential nodular tracheal and central bronchial thickening. Flexible bronchoscopy showed papilomatous-like lesions, lending to initial concern for tracheobronchial papillomatosis with parenchymal involvement. Ulcerated tracheal mucosa with fibropurulent exudate was seen on biopsies. Pathology was negative for papillomatosis, viral cytopathic changes, evidence of fungal organisms or malignancy. The patient was empirically started on high-dose corticosteroids with rapid improvement of her respiratory status. Extensive workup for alternative infectious or autoimmune etiologies was negative. The patient remained symptom free at discharge with plans to undergo repeat chest CT four weeks after initiation of steroids to monitor for resolution of pulmonary findings. Extraintestinal manifestations of IBD develop in 21-41% of patients with a higher incidence in those with UC as compared to Crohn's Disease (CD). Pulmonary involvement is rare, occurring in only 0.21% of patients and is often misdiagnosed as asthma on initial presentation. A wide variety of bronchoscopic findings have been reported including severe tracheal narrowing, ulcerated mucosa, a cobblestone appearance and pus. Systemic corticosteroids led to rapid improvement in symptoms in most reported cases of large airway involvement. As seen with the above case, pulmonary manifestations of IBD can be fatal but prompt diagnosis and early intervention can lead to improved outcomes.Figure: Tracheal papilomatous-like lesions found on flexible bronchoscopy in a patient who presented with stridor.Figure: Diffuse tracheobronchial thickening consistent with tracheobronchitis in a patient with inflammatory bowel disease.