Division of Rheumatology, Department of Medicine, Hospital for Special Surgery, New York, NY, USA.
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.
Curr Allergy Asthma Rep. 2020 Jul 9;20(10):56. doi: 10.1007/s11882-020-00953-1.
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.
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. This is a narrative review that provides a comprehensive overview of localized granulomatosis with polyangiitis and current treatment options.
肉芽肿性多血管炎是一种常见的原发性系统性血管炎，通常以上呼吸道、肺部和肾脏受累的典型三联征为特征。上呼吸道和下呼吸道受累是肉芽肿性多血管炎患者的特征表现，有时代表首发症状，有时则是唯一表现。本文综述的目的是总结 GPA 局限性疾病的各种临床表现及其治疗方法。
90%的患者存在鼻窦疾病。耳部和眼部受累也很常见。虽然喉部和气管疾病不太常见，但与显著的发病率相关，并可能具有治疗挑战性。临床医生需要意识到这些局限性 GPA 表现，因为它们可能是在没有其他全身表现的情况下出现的疾病特征。局限性 GPA 的治疗包括免疫抑制和手术干预特定表现。包括风湿病学家、耳鼻喉科医生和眼科医生在内的专家之间的合作，对于确保患者获得最佳结果往往至关重要。这是一篇叙述性综述，全面概述了局限性肉芽肿性多血管炎及其当前的治疗选择。
