Late Endobronchial Wegener's Granulomatosis

Abstract

CASE A 58-year-old man with a known diagnosis of Wegener's granulomatosis (WG) since 1990 was treated with prednisone and Cytoxan for 3 to 4 years until he was thought to be in remission. Despite the therapies, he continued to have shortness of breath and bilateral recurrent pneumonia. Flexible bronchoscopy revealed 20%, 90%, and 100% stenosis of the subglottic trachea, truncus intermedius (Fig. 1), and lingular bronchus respectively.FIG. 1.: Diffuse narrowing and multiple strictures involving the entire truncus intermedius in a patient with Wegener's granulomatosis.DISCUSSION WG is an idiopathic, multisystem disease characterized by necrotizing granulomatous inflammation and vasculitis that involves primarily the upper and lower respiratory tracts with associated glomerulonephritis. 1–5 Actually, WG can involve the entire respiratory tract from the nasal septum to the pleura. 6 A limited form, with a clinical finding isolated to the upper respiratory tract or the lungs, occurs in approximately one fourth of cases. 1,5,7,8 The lung is the most frequent and sometimes the only organ involved. It has been suggested that the pulmonary involvement results from enhanced exposure to inhaled substances and/or a respiratory infection combined with unique susceptibility factors found in the host. 1,9 Approximately 85% of patients develop lung involvement during the course of the disease, 6,10,11 and 45% have lung involvement at presentation. 11 Two thirds of the former group have respiratory symptoms with active disease, whereas the remaining patients are asymptomatic despite an abnormal chest radiograph. 6 In WG, pulmonary symptoms include cough, hemoptysis, and pleuritis. 1,6 The radiographic findings usually include nodules that may cavitate and are seen in half the patients, as well as alveolar and pleural opacities. 1,6 Also, lobar and segmental infiltrates or atelectasis secondary to endobronchial disease have been reported. 6,8,12 Hemoptysis is the most frequent symptom, which leads patients to undergo bronchoscopy frequently (55–59%), revealing gross endobronchial abnormalities such as isolated hemorrhage, airway inflammation, ulcerations, bronchial stenosis, and pseudotumors. 4,6,11,13 Inflammation and stenosis of the endobronchial airways has been found to occur in at least 15% of patients with lung involvement. 9,13 One of the locations where WG can cause irreversible damage is the tracheobronchial tree, which could be potentially life-threatening. 2,13 The incidence of tracheal stenosis has been reported to be 16%. 2,14 The incidence of subglottic involvement has also been reported with similar frequency. 2,5,6,14–17 Subglottic stenosis sometimes develops during the course of the disease and is a result of circumferential inflammation, edema, and fibrosis that typically extends for 3 to 4 cm below the vocal cords, 16 and results in long-term morbidity. Our patient presented with symptoms of airway obstruction and was found to have subglottic stenosis as well as stricture involving the entire truncus intermedius. Another form, called ulcerating tracheobronchitis, which is characterized by focal or diffuse airway narrowing as a result of the inflammation and edema, 6,16 can accompany WG. Also, hilar adenopathy and mediastinal masses are not uncommon with WG. 10 The diagnosis of WG relies heavily on pathologic documentation of granulomatous inflammation, necrosis, and vasculitis. 1,6,9,10,18,19 Open lung biopsy is the most sensitive method for establishing the diagnosis because it demonstrates necrotizing granulomatous vasculitis in 70% of patients. 1,3,18 Endobronchial lung biopsy has a role in the diagnostic evaluation of WG when it exhibits ulcerative, exophytic, or stenotic tracheobronchial lesions. 1,3,5,6,13,18–21 Therapeutic interventions for endobronchial WG depend on the presence or absence of airway inflammation. Airway inflammation suggests acute disease and may respond to systemic treatment. 9,15 Conversely, airway stenosis (subglottic or tracheal) may suggest a burned-out, late, irreversible aftermath of WG. Under the circumstances, interventions such as laser photoresection, dilation, and stent placement may be necessary. 9,13,15,16 Our patient underwent endobronchial photoresection using the Nd:YAG laser, balloon bronchoplasty, and mitomycin applications to the treatment areas.