INTRODUCTION: Up to 40 % of individuals with ANCA associated granulomatosis vasculitis (formerly known as Wegener’s granulomatosis) have airway involvement. Airway involvement can progress independent of other pulmonary manifestations and rarely responds to systemic treatment alone. We describe a case of advanced airway involvement secondary to Wegner’s treated successfully with bronchoscopic intervention.
CASE PRESENTATION: This is a case of a 74 year old female with a diagnosis of Wegener's granulomatosis. She originally presented with hemoptysis, lung nodules and hematuria. The patient was successfully treated with methylprednisolone and cyclophosphamide. For maintenance therapy she was transitioned to mycophenolate mofetil and weaned off of steroids. Three months later, she had a recurrence of hemoptysis, weight loss, and hematuria. This episode responded to the addition of 60 mg of prednisone and an increase in the mycophenolate to 2 g a day. Over the following three months she developed progressive dyspnea, which eventually lead to her presentation to the emergency department with respiratory distress and stridor. The airway CT scan demonstrates a widely patent trachea and a narrowed right main stem bronchus. A bronchoscopy was performed with the patient awake to avoid further airway compromise. There was severe complex stenosis in the right and left main stem bronchi. She was initiated on high dose steroids and Rituximab. A staged bronchoscopic intervention was planned as follows; electocautry followed by balloon dilation and Mitomycin C application to the left main bronchus. A separate procedure was then performed to address the right main stem bronchus. Dexamethasone 2 mls(8mg) was injected into the stenotic area of the right upper lobe and right main stem bronchus using a 19 gauge TBNA needle. Three days later the area was dilated with a balloon to achieve patency of the right main stem bronchus and right upper lobe. Mitomycin C was then applied to the area. A follow up bronchoscopy two weeks post intervention demonstrated complete resolution of all obstruction and normal appearance of airways.
DISCUSSION: Airway involvement secondary to ANCA associated granulomatosis is difficult to treat and can cause life threatening airway obstruction. We describe a case of severe airway involvement treated successfully with bronchoscopic interventions combined with systemic therapy. We recommend a staged procedure working on the left and right main stem airways independently in case of increased inflammation and swelling from the bronchoscopic procedure further compromising the airway. Mitomycin C has antifibrotic properties that are though to delay re-stenosis however there is no data to support this.
CONCLUSIONS: Advanced airway involvement from ANCA associated granulomatosis vasculitis is optimally treated with systemic therapy in combination with local bronchoscopic treatment.
Reference #1 Polychronopoulos, et al. Rheum Dis Clin N Am 33 (2007) 755-775
DISCLOSURE: The following authors have nothing to disclose: Renelle Myers, Andres Sosa, Gaetane Michaud
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