Fulminant obliterative tracheobronchitis as an initial presentation of ulcerative colitis

Introduction: Extra-intestinal manifestations of ulcerative colitis involving the pulmonary system are uncommon. One such rare manifestation is large airway involvement with ulceration, squamous metaplasia of the epithelium and exuberant granulation tissue described as obfiterative tracheobronchitis. We present a case of fulminant tracheobronchitis as the initial presentation of ulcerative colitis. Case Presentation: A physically active 50-year-old African-American male with borderline hypertension presented to a local hospital with three days of progressive non-productive cough, dyspnea, bloody diarrhea and fever. There was no smoking history or exposure to toxins. He was found to be in severe respiratory distress, with hypoxemic, hypercarbic respiratory failure with a pulmonary exam notable for stridor and diffuse wheezing. Chest radiograph showed dense consolidation and left upper lobe volume loss. The patient was endotracheally intubated and was difficult to ventilate, requiring high peak inspiratory pressures. Bilateral pneumothoraces occurred that were treated with chest tube thoracostomies. The decision was made to transfer the patient to our facility for further care. However, en route the patient suffered a prolonged cardiac arrest secondary to hypoxemia but was successfully resuscitated. Upon arrival the patient remained difficult to ventilate, showing poor dynamic compliance. An initial arterial blood gas showed acidemia, profound hypercarbia and a partial pressure of oxygen of 96 mm Hg while inspiring 100% oxygen. Laboratory studies were remarkable for a white blood cell count of 29,000 without eosinophilia, normal hemoglobin level and an elevated lactate dehydrogenase of 911 mg/dl (upper limits of normal 618 mg/dl). Chest and abdomen computerized tomography showed circumferential tracheal thickening, narrowing of both mainstem bronchi, left upper lobe collapse and diffuse colonic bowel wall thickening. Bronchoscopy showed pale, necrotic and narrowed central airways below the endotracheal tube with an inability to pass the bronchoscope through either mainstem bronchus. Mucosal biopsies showed fibrinopurulent debri with reactive epithelial cells and many polymorphonuclear cells. Stains and cultures for organisms were negative. A colonoscopy revealed markedly inflamed and friable mucosa with biopsies showing ulcerated granulation tissue and crypt abscesses consistent with ulcerative colitis. The patient was empirically treated with broad spectrum antibiotics along with high dose corticosteroids. The respiratory status improved markedly with increased tidal volumes on pressure control ventilation, improved oxygenation and near resolution of the chest radiographic abnormalities. A repeat bronchoscopy, after 48 hours of therapy, showed marked improvement in the central airways with visualization of all pulmonary segments. On hospital day number four the patient had acute worsening of his respiratory status. Emergent bronchoscopy showed a large tissue mass occluding both mainstem bronchi which was extricated using a wire basket extractor. Pathologic examination of the specimen showed it to be 7 centimeters, bifurcated and composed or fibrin and necrotic debris thought to be sloughed tissue of the large central airways. Because of progressive abdominal distension and radiologic evidence of bowel wall thickening, the patient underwent colectomy. Histology showed severe active colitis. The patient's post-operative course was uncomplicated with continued improvement in his respiratory and gastrointestinal status; however the patient manifested persistent sequel of severe anoxic encephalopathy. Discussion: Although previously reported, tracheobronchitis in relation to ulcerative colitis is extremely rare. To our knowledge this is the first report of a fulminant case of tracheobronchitis that was part of the patient's initial presenting symptoms of ulcerative colitis. Our patient showed a dramatic response to corticosteroids as evidenced by improved ventilation and gross appearance of the airways on serial exams. Conclusion: Obliterative tracheobronchitis can be a catastrophic manifestation of ulcerative colitis and should be in the differential for acute respiratory failure with airflow obstruction.