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Journal ArticleDOI

Otolaryngological progression of granulomatosis with polyangiitis after systemic treatment with rituximab.

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TLDR
While rituximab has been shown to be noninferior to cyclophosphamide with respect to remission from systemic GPA, these patients continue to have chronic otolaryngological manifestations of their disease and must continue to play a supportive role throughout their maintenance period.
Abstract
ObjectiveRituximab is used for the treatment of granulomatosis with polyangiitis (GPA), historically known as Wegener’s granulomatosis. However, the otolaryngological progression of GPA after systemic treatment with rituximab (Rituxan) is unclear. We therefore examined the disease sequelae of patients with GPA who were treated with rituximab.Study DesignCase series with chart review.SettingTertiary care medical center.Subjects and MethodsPatients with a diagnosis of GPA who were treated with rituximab between 2006 and 2012 were included in this study. Systemic and otolaryngological symptomatology, prednisone usage, and procedural interventions following B-cell depletion were analyzed.ResultsWe identified 11 patients who met our inclusion criteria. The average length of follow-up after treatment with rituximab was 23.5 months. After treatment with rituximab, there was a significant decrease in daily prednisone dose at 3, 12, and 18 months postinfusion (P < .05). However, there was no observed improvement i...

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Journal ArticleDOI

Rituximab for induction and maintenance therapy in granulomatosis with polyangiitis (Wegener's). Results of a single-center cohort study on 66 patients.

TL;DR: It is concluded that in this single-center cohort, RTX associated with glucocorticoid treatment adapted for disease severity appeared to induce remission effectively in GPA patients, and maintenance treatment with low doses of RTX in a routine time-based protocol was safe and associated with low rates of relapse on treatment.
Journal ArticleDOI

Granulomatosis With Polyangiitis in Otolaryngologist Practice: A Review of Current Knowledge.

TL;DR: In clinical practice diagnosis, the presence of distinctive ANCA antibodies and biopsy of affected organ are crucial, and GPA must be differentiated from neoplastic, infectious or inflammatory ulcerative lesions of the head and neck.
Journal ArticleDOI

Clinical aspects of granulomatosis with polyangiitis affecting the head and neck

TL;DR: Early and sufficient systemic therapy for all HN-GPA patients is suggested and Nasal mucosal “blind” biopsy should be performed in patients with elevated auto-antibodies but lacking clinical head and neck manifestations.
Journal ArticleDOI

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

TL;DR: It is reported that two females aged 40 and 52 years, admitted to the hospital with a subglottic tracheal stenosis, responded to rituximab and required a tracheostomy.
References
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Journal ArticleDOI

Structured clinical assessment of the ear, nose and throat in patients with granulomatosis with polyangiitis (Wegener’s)

TL;DR: The pattern and frequency of clinical ENT manifestations in GPA have been described in a large patient cohort and will form the basis of an objective scoring system to measure disease activity in the ENT system.
Journal ArticleDOI

Analysis of Wegener's granulomatosis responses to rituximab: current evidence and therapeutic prospects.

TL;DR: Data on small numbers of patients with refractory and/or relapsing Wegener’s granulomatosis support very good overall outcomes with rituximab, an anti-CD20 biotherapy targeting B cells, in combination with ongoing immunosuppressants, apparently independently of antineutrophil cytoplasm antibody (ANCA) status.
Journal ArticleDOI

Progression and management of Wegener's granulomatosis in the head and neck

TL;DR: To describe the otolaryngologic presentation and natural history of granulomatosis with polyangiitis (GPA), previously known as Wegener's granulOMatosis, and to compare otolARYngologic outcomes of patients with systemic GPA to those with a limited form of GPA confined to the head and neck.
Journal ArticleDOI

Advances in Therapy for ANCA-Associated Vasculitis

TL;DR: This article reviews seminal studies from the past decade that have contributed to the current standard of care in the anti-neutrophil cytoplasmic antibody-associated vasculitides and suggests plasma exchange may be an effective adjuvant therapy.
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