Clinical features and therapeutic management of subglottic stenosis in patients with Wegener’s granulomatosis

Abstract

The objective of the study was to evaluate the clinical features, response to treatment, and long-term outcome of subglottic stenosis (SGS) in a series of patients diagnosed as having Wegener's granulomatosis (WG) at a single institution. Subglottic stenosis developed in 6 out of 51 (11.7%) patients, in four of them in the absence of other features of active disease, and was the symptom that leads to WG diagnosis in three cases. In two cases, SGS began while the patients were receiving systemic immunosuppressive therapy for disease activity involving other sites. PR3-ANCAs were positive in four cases. An urgent tracheostomy was needed in two patients. Four patients achieved SGS clinical remission on standard treatment with glucocorticoids and cyclophosphamide, but three of them experienced repeated local relapses and required additional immunosuppressive therapy and mechanical dilations. In one case, a local relapse was successfully managed with endotracheal dilation of the stenotic segment and intralesional injection of a long-acting corticosteroid plus mechanical dilation of the stenotic segment (ILCD) without adding supplemental immunosuppressant drugs. Two patients with isolated SGS were also successfully managed with ILCD alone and did not require the institution of systemic immunosuppressive therapy. One patient underwent open surgical repair when the disease was under control. Our data suggest that Subglottic stenosis often occurs or progresses independently of other features of active WG, and that ILCD may be a safe alternative to conventional immunosuppressive therapy in patients who develop SGS in the absence of other features of active disease, allowing reducing the treatment-related toxicity.

Predicament of classification: Multisystem small vessel vasculitis with cresentic Glomerulonephritis

“…Tracheal and pulmonary disease can be seen in the majority of ANCA associated vasculitis, (AAV) [16,[18][19][20]. Tracheal or subglottic stenosis, pulmonary consolidation or ibrosis, and pulmonary arterial hypertension are some of the manifestation of the disease [18,21].…”

Estenosis subglótica y granulomatosis con poliangeítis (Wegener) en dos casos: Report of two cases

“…El manejo va a depender de si la ESG ocurre y progresa de manera independiente con la actividad de la enfermedad, o si es parte de ella y es en el primer caso donde el tratamiento local está indicado por sobre el tratamiento convencional o sistémico, y cuando es parte de la actividad de la enfermedad ambos tratamientos son utilizados [6][7][8][9][14][15][16][17] . El 25% responde a tratamiento inmunodepresor.…”

“…Existen algunos reportes de casos que apoyan la utilización de rituximab en este contexto, como en nuestro primer paciente 18,19 . Algunos autores proponen dejar al paciente con corticoides inhalados, tipo fluticasona en dosis de 440 a 880 mg dos veces cuando la dosis de corticoides sistémicos se disminuye por debajo de 30 mg [6][7][8][9][14][15][16] .…”

“…La elección del tipo y tiempo del procedimiento depende de la experiencia del equipo tratante y sólo debe ser hecha en centros de referencia. Las diferentes terapéuticas disponibles hacen que la realización de traqueostomía se demore o no sea necesaria, a pesar de que en algunas series hasta 40% de los pacientes la requieren por progresión irreversible de la estenosis [14][15][16][17] .…”

Predicament Of Classification: Multisystem Small Vessel Vasculitis With Cresentic Glomerulonephritis

“…Tracheal and pulmonary disease can be seen in the majority of ANCA associated vasculitis, (AAV) [16,[18][19][20]. Tracheal or subglottic stenosis, pulmonary consolidation or fibrosis, and pulmonary arterial hypertension are some of the manifestation of the disease [18,21].…”