Abstract
Objectives
To describe the presentation and clinical course of subglottic stenosis (SGS), in particular the development of concurrent airway lesions, in patients with Granulomatosis with Polyangiitis (Wegener’s) (GPA).
Materials and methods
Retrospective review of clinical data from all patients presenting to our institution from 2000 to 2012 with SGS and GPA.
Results
Thirty-five patients were identified. The average age at diagnosis was 33 years old. Eleven patients (31%) presented with SGS as part their initial manifestation of GPA. The remaining patients developed SGS later, at a median of 2.5 years from diagnosis (range 6 months to 14 years). Twelve patients (34%) were noted to have multilevel airway involvement. Seven patients (20%) had documentation of cricoarytenoid joint fixation and vocal cord immobility. This was typically progressive in nature and occurred at an average of two years following the diagnosis of SGS. Six patients (17%) had mid/distal tracheal stenosis and four (11%) had bronchial stenosis. The majority of patients (86%) had evidence of concurrent sinonasal involvement, ten patients (29%) had evidence of otologic involvement and eight (23%) had ocular involvement.
Conclusions
Cricoarytenoid joint fixation and distal stenosis occur not infrequently in patients with GPA and SGS, resulting in progressive multilevel airway stenosis in about one third of patients. It is critical to identify multilevel stenosis when managing the airways of these patients.
1
Introduction
Granulomatosis with polyangiitis (Wegener’s) (GPA) is a multisystem disease characterized by necrotizing granulomatous inflammation and vasculitis affecting approximately 3 in 100,000 individuals in the United States . GPA classically involves the upper airway, lung and kidneys. Diagnosis is typically made by the presence of necrotizing granulomatous inflammation of the upper respiratory tract and small to medium size vessel vasculitis on histology and/or anti-neutrophil cytoplasmic antibody (ANCA) serologies.
Otolaryngologic manifestations occur in 87%–92% of patients with GPA, often as the presenting symptom . The most common head and neck manifestation is sinonasal disease, occurring in 85% of patients, followed by otitis media (44% of patients) . Subglottic stenosis (SGS) is a less common, but a life-threatening manifestation of GPA, especially if not identified and correctly managed. The incidence of subglottic stenosis in GPA has been estimated to be 8%–23% and occurs more commonly in younger patients . It may occur in isolation as the presenting symptom or as a late-stage manifestation of disease . Stenosis is often limited to the subglottis/proximal trachea, but may extend into the distal trachea and bronchi. Involvement of the glottic and supraglottic larynx may also occur, in particular cricoarytenoid joint fixation, but its incidence is not well defined . In general, the course of SGS has been found to run independently of the systemic disease course of GPA .
In this study we aim to characterize the clinical presentation of SGS in GPA, in particular the incidence of multilevel airway disease, including distal tracheal, bronchial and glottic involvement, which has not been well established.
2
Materials and methods
The Institutional Review Board at St. Luke’s-Roosevelt Hospital Center (Now Mt. Sinai-Roosevelt Hospital) approved this retrospective study. The records of all patients presenting to the senior author (RL) at the Head and Neck Surgical Group at St. Luke’s-Roosevelt Hospital Center from 2000 to 2012 with a diagnosis of GPA and SGS were reviewed. Patients were included in the study if they had significant subglottic stenosis, defined as requiring at least one surgical intervention and compelling evidence of GPA by histological findings and/or positive ANCA serologies. SGS and proximal tracheal stenosis were grouped together when describing the airway lesions. Patients in whom the diagnosis of GPA was uncertain or whose medical records were incomplete or did not include information on the initial presentation of GPA were excluded. Clinical history, demographics, age at diagnosis, time to development of SGS, sites of GPA involvement, physical exam findings, endoscopy findings, pathology and laboratory studies were reviewed and compiled into a database.
2
Materials and methods
The Institutional Review Board at St. Luke’s-Roosevelt Hospital Center (Now Mt. Sinai-Roosevelt Hospital) approved this retrospective study. The records of all patients presenting to the senior author (RL) at the Head and Neck Surgical Group at St. Luke’s-Roosevelt Hospital Center from 2000 to 2012 with a diagnosis of GPA and SGS were reviewed. Patients were included in the study if they had significant subglottic stenosis, defined as requiring at least one surgical intervention and compelling evidence of GPA by histological findings and/or positive ANCA serologies. SGS and proximal tracheal stenosis were grouped together when describing the airway lesions. Patients in whom the diagnosis of GPA was uncertain or whose medical records were incomplete or did not include information on the initial presentation of GPA were excluded. Clinical history, demographics, age at diagnosis, time to development of SGS, sites of GPA involvement, physical exam findings, endoscopy findings, pathology and laboratory studies were reviewed and compiled into a database.
3
Results
A total of thirty-five patients, twenty-four female and eleven male, with GPA and SGS were identified. The average age at diagnosis of GPA was 33 years old. Diagnosis was made by tissue biopsy alone in six patients, ANCA serologies in nine patients and a combination of both in twenty patients. The most common site for a diagnostic biopsy was the lung (11 patients), followed by nasal mucosa (9 patients) and subglottic/tracheal mucosa (4 patients) ( Table 1 ).
