The patient was a 16-year-old boy who was admitted with a 1-month history of rhinorrhea, headache, poor general condition, fever, dyspnea and purulent discharge. He had an infraorbital edema and nasal deformity with scabs. A chest radiograph was normal and computed tomography (CT) revealed opacification of the maxillary sinus and ethmoidal cells. He had 22,660leukocytes/mm3 (85.7% neutrophils), 1,174,000platelets/mm3, C-reactive protein was 18.8mg/dL (normal value<0.5) and normal renal function. He did not improve with antibiotic therapy and, thus, the studies were repeated: a second CT scan of the paranasal sinuses showed lysis of the wall of left maxillary sinus and resorption of turbinates, chest radiograph revealed bilateral alveolar infiltrates and chest CT showed multiple cavitated nodules. He was positive for antineutrophil cytoplasmic antibodies (ANCA) with a titer of 1:640, cytoplasmic-ANCA pattern, anti-proteinase 3 (PR3) antibodies >1607IU and complement levels were normal. A biopsy of a turbinate revealed necrosis, infiltrates with polymorphonuclear cells and lymphocytes, and necrotic vessels. Bronchoscopy revealed friable mucosa and mucopurulent secretions without stenosis and Staphylococcus aureus was isolated in the nasal exudate. The diagnosis was GPA-like vasculitis, and immunosuppressive therapy was begun with 250mg daily of intravenous methylprednisolone for 3 days, followed by oral prednisone in a tapered dose starting at 45mg/day, 500mg intravenous pulses of cyclophosphamide every 15 days for 3 months, as well as omeprazole, vitamin D and cotrimoxazole.

Despite treatment, the patient became progressively worse. After 9 months, he had dyspnea on minimal exertion, stridor and hemoptysis. Spirometry revealed a forced expiratory volume in 1s (FEV1) of 3.33 and a FEV1/forced vital capacity (FVC) ratio of 56.85%. A subsequent spirometry disclosed severe airflow limitation, with a FEV1 of 2.07 and a FEV1/FVC ratio of 48.46%. Another CT scan showed an improvement in the pulmonary lesions, without BS, and laryngeal endoscopy ruled out SGS. Finally, bronchoscopy was repeated and disclosed several web-like membranous stenoses at the level of the lower and left upper lobes. The patient underwent mechanical dilatations, which achieved clinical improvement. He was treated with a 1g dose of rituximab, which was discontinued as he had a severe allergic reaction, megadoses of steroids and, again, two 500mg pulses of cyclophosphamide every 15 days. Nevertheless, the BS recurred, with normalized acute-phase reactants, and he required balloon dilatations and endoscopic treatment (Fig. 1).

Fig. 1.

Bronchoscopic image showing a web-like concentric membranous stenosis that crosses the entrance to the base of right lower lobe, with a minimal central aperture that impedes the passage of the bronchoscope to the distal region. In left bronchus, the same stenosis but with a larger central aperture.

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There is little information on the management of BS in GPA. Although they are associated with SGS, they can develop in the absence of that lesion. Girard et al.6 recently reported their experience in 10 French patients with BS. They presented with dyspnea and hemoptysis, but without stridor. In 6 cases, BS was an incidental finding after the performance of a CT scan or fiberoptic bronchoscopy for another reason. In general, the diagnosis was reached within the first 3 months of the disease. In GPA, BS are usually multiple, predominantly involving the left bronchus, generally in the upper lobe, and the tendency for relapses was independent of the treatment. It is unusual that 8 of the 9 patients who had recurrences were in systemic remission as they were receiving immunosuppressive therapy, with a mean of 3 recurrences per patient. One of them had 12 relapses.

Our patient had a web-like stenosis (WLS) or “in diaphragm”, that is believed to develop due to an exaggerated inflammatory response and hypertrophy of the mucosa, and formation of fibromembranous tissue that comes to progressively occlude the bronchial lumen.7–9 Web-like stenoses have been related to infections, traumatic injury and local or systemic inflammatory processes. Three cases of WLS have been reported with GPA and one with microscopic polyangiitis,8 and isolated cases with vasculitis, sarcoidosis, amyloidosis, Behçet's disease and ulcerative colitis.9,10 In contrast to non-web-like BS, lung CT is normal. Thus, only pulmonary functions tests and bronchoscopy enable the establishment of the diagnosis. The prognosis is poor, requiring dilatations using a rigid bronchoscope, endobronchial laser, local steroid injections and even the placement of stents in the case of proximal stenoses. Treatment and follow-up should be discussed with bronchoscopists or thoracic surgeons. The purpose is to perform the available endobronchial technique and optimize immunosuppressive therapy.