Role of thoracic surgery and interventional bronchoscopy in Wegener's granulomatosis

Ann Thorac Surg. 2002 Dec;74(6):1948-52. doi: 10.1016/s0003-4975(02)04027-4.


Background: Wegener's granulomatosis (WG) is defined as granulomatous vasculitis affecting small and medium-sized arteries and veins. Histologically, inflammatory changes with infiltration of the endothelium, fibrinoid necrosis, and formation of necrotizing granulomas are found. Pulmonary involvement is one of the cardinal features of WG and occurs in 85% of patients during the course of disease. Surgery is often required for both diagnosis and therapy.

Methods: Fifteen consecutive patients are presented to illustrate the spectrum of surgical interventions in WG.

Results: In 8 patients open lung biopsy, wedge resection, or segmental resection for hitherto undiagnosed infiltrate revealed WG. Eight patients presented with tracheal stenosis and all 8 underwent repetitive tracheoscopic dilation. Five patients presented with subglottic stenosis without any signs of pulmonary manifestation. All patients underwent tracheoscopic dilation, 3 in conjunction with glucocorticoid injection therapy. After multiple dilations, 1 patient still had destructing ulcerative tracheitis in which total stenting maintained airway patency. One patient received subglottic tracheal resection prior to multiple dilations; another patient, because of expiratory tracheal collapse, underwent stabilization of the membranous part of the trachea and the large bronchi with a polytetrafluoroethylene implant.

Conclusions: Surgical lung biopsy in numerous patients established the final diagnosis. Thoracic surgery including bronchologic measures such as bouginage and stenting, however, also has a place in the long-term management of WG.

MeSH terms

  • Adult
  • Aged
  • Biopsy / methods
  • Bronchoscopy*
  • Dilatation / methods
  • Female
  • Granulomatosis with Polyangiitis / pathology*
  • Granulomatosis with Polyangiitis / surgery*
  • Humans
  • Lung Diseases / pathology*
  • Lung Diseases / surgery*
  • Male
  • Middle Aged
  • Thoracoscopy*
  • Tracheal Stenosis / complications