Service d'ORL et chirurgie cervico-faciale, CHU de Clermont-Ferrand, 58, rue Montalembert, Clermont-Ferrand, France.
Service d'ORL et chirurgie cervico-faciale, CHU de Clermont-Ferrand, 58, rue Montalembert, Clermont-Ferrand, France.
Eur Ann Otorhinolaryngol Head Neck Dis. 2019 Nov;136(6):509-511. doi: 10.1016/j.anorl.2019.05.008. Epub 2019 Aug 5.
Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis of small and medium-sized vessels comprising inflammation of the vessel wall and perivascular and extravascular granulomas, frequently presenting in the form of chronic sinusitis.
We report the case of a 27-year-old man who presented with very painful acute frontal sinusitis that was managed medically and surgically. The symptoms rapidly recurred despite treatment and CT scan demonstrated diffuse thickening of the sinus mucosa. Anti-proteinase 3 ANCA were positive. Biopsy of a pulmonary nodule confirmed the diagnosis of GPA. The patient was treated with corticosteroids in combination with rituximab, resulting in improvement of the clinical, laboratory and CT signs.
In the presence of persistent, acute, localized sinusitis despite appropriate treatment, associated systemic signs and/or the presence of other signs suggestive of GPA, the ENT surgeon must request a targeted work-up. In the absence of treatment, GPA can be fatal within a few months. However, with currently available treatment, remission is obtained in 80% of cases with a 75% 10-year survival rate.
肉芽肿性多血管炎（GPA）是一种小血管和中等大小血管的坏死性血管炎，包括血管壁炎症和血管周围及血管外肉芽肿，常表现为慢性鼻窦炎。
我们报告了一例 27 岁男性患者，表现为非常疼痛的急性额窦炎，经药物和手术治疗。尽管治疗后症状迅速复发，且 CT 扫描显示窦粘膜弥漫性增厚。抗蛋白酶 3 抗中性粒细胞胞浆抗体阳性。肺结节活检证实 GPA 的诊断。患者接受了皮质类固醇联合利妥昔单抗治疗，临床、实验室和 CT 征象均有所改善。
在适当治疗后仍持续存在急性、局部鼻窦炎，伴有全身系统表现和/或存在其他提示 GPA 的表现时，耳鼻喉科医生必须进行有针对性的检查。如果不治疗，GPA 在几个月内可能致命。然而，目前的治疗方法可使 80%的病例缓解，10 年生存率为 75%。
