Recurrent Cryptogenic Ulcerative Tracheobronchitis 6 Years After Lung Transplantation: Case Report.

BACKGROUND: Tracheobronchitis is an uncommon pattern of airway disease and is typically caused by infection, inhalation injury, infiltrative disorders and more rarely, systemic autoimmune disease. Rarer again is the development of bronchial webbing following tracheobronchitis. The underlying aetiology is usually identified through biopsy, culture or a detailed clinical history. We present a case of cryptogenic ulcerative tracheobronchitis with bronchial webbing being successfully managed by bilateral lung transplantation that has, however, been complicated by recurrence. CASE PRESENTATION: In 2019, Crowhurst et al. detailed the case of a patient who presented with dyspnoea and haemoptysis. Bronchoscopy revealed ulcerative tracheobronchitis and he subsequently formed recurrent web-like stenoses of the central bronchi, leading to rapidly progressive type 2 respiratory failure; he received bilateral sequential lung transplantation for definitive management. At the time of transplant, the presumed diagnosis was an inhalation injury that occurred while travelling in Vietnam, or exposure to the organophosphate pesticide Malathion several days prior. The explanted lung histology revealed ulcerating bronchitis with intense histiocytic infiltrates, bronchial luminal fibrosis and obstruction with scant, poorly formed granulomas. The patient thrived with stable lung function and had minimal complications initially post-transplantation. However, 6 years after lung transplantation and without re-exposure, the patient re-developed ulcerative tracheobronchitis, which shared similar histopathologic features to airway involvement of Crohn's disease. Gastrointestinal or other organ system involvement of Crohn's disease was not established despite extensive investigations. A decision for phenotypic treatment with the TNF-α inhibitor, infliximab, was made with subsequent rapid improvement in the tracheobronchitis. CONCLUSION: This is the first case documented in the literature of ulcerative tracheobronchitis followed by intense bronchial web-like stenoses caused by an unspecified autoimmune disease, treated by bilateral lung transplantation. The disease recurred after 6 years and responded to phenotypic management with the TNF-α inhibitor, infliximab. The patient will require careful monitoring given the risk of augmented immunosuppression and unknown disease trajectory.