Localized Granulomatous with Polyangiitis (GPA): Varied Clinical Presentations and Update on Treatment
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AUTOIMMUNITY (T TARRANT, SECTION EDITOR)
Localized Granulomatous with Polyangiitis (GPA): Varied Clinical Presentations and Update on Treatment Sebastian E. Sattui 1 & Lindsay Lally 2
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review Granulomatosis with polyangiitis is a primary systemic vasculitis commonly described with the typical triad of upper airway, lung, and kidney involvement. Upper and lower airway involvement is characteristic in patients with granulomatosis with polyangiitis and can sometimes represent the initial or in some instances the sole manifestation. The objective of this review is to summarize the various clinical manifestations of localized disease in GPA and their treatment. Recent Findings Sinonasal disease is seen in up to 90% of patients. Otologic and ocular involvement is also commonly seen. Laryngeal and tracheal disease although less common is associated with significant morbidity and can be therapeutically challenging. Clinicians need to be aware of these localized GPA manifestations as they may be presenting disease features in the absence of other systemic findings. Treatment of localized GPA involves both immunosuppressive and surgical interventions for specific manifestations. Collaboration between specialists including rheumatologists, otolaryngologists, and ophthalmologists is often crucial to ensure optimal outcomes for patients. Summary This is a narrative review that provides a comprehensive overview of localized granulomatosis with polyangiitis and current treatment options. Keywords Granulomatosis with polyangiitis . ANCA . Rituximab
Introduction As one of the antineutrophil cytoplasmic antibody (ANCA)– associated vasculitides, Granulomatosis with polyangiitis (GPA) is a multisystem disease characterized by small to medium vessel necrotizing vasculitis with granulomatous inflammation. GPA, previously known as Wegener’s granulomatosis, has a predilection for the respiratory epithelium of both the upper and lower airways and the kidneys. Prevalence of GPA between 3.2 and 9.1 cases per 100,000 has been reported in the USA; however, a more recent population study has This article is part of the Topical Collection on Autoimmunity * Lindsay Lally [email protected] 1
Division of Rheumatology, Department of Medicine, Hospital for Special Surgery, New York, NY, USA
2
Division of Rheumatology, Department of Medicine, Weill Cornell Medicine, Scleroderma, Vasculitis and Myositis Center, Hospital for Special Surgery, 525 East 71st Street, 7th Floor, New York, NY 10021, USA
suggested an increasing prevalence (21.8 per 100,000) and an increase annual incidence of 1.3 cases per 100,000 [1–3]. While GPA has several overlapping features with microscopic polyangiitis (MPA), the presence of granulomatous inflammation in addition to necrotizing vasculitis is the key histopathologic distinction. Phenotypically, the granulomatous inflammatory lesions distinguish GPA from MPA; these manifestations include rhinosinusitis, subglottic stenosis, orbita
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