Wei Jinlong, Zhao Qin, Yao Min, Meng Lingbin, Xin Ying, Jiang Xin
Department of Radiation Oncology.
Jilin Provincial Key Laboratory of Radiation Oncology & Therapy, The First Hospital of Jilin University.
Medicine (Baltimore). 2021 Jan 22;100(3):e22794. doi: 10.1097/MD.0000000000022794.
Granulomatosis with polyangiitis (GPA) is a chronic systemic vasculitis characterized by necrotizing granulomatous vasculitis. The disease mainly affects the middle and small blood vessels and mainly occurs in the upper respiratory tract (nose and paranasal sinuses), lower respiratory tract (lungs), and kidneys. Disease occurrence in the eyelid area is relatively rare. The standard GPA treatment is combination therapy with adrenocortical hormone and immunosuppressants. Radiotherapy as a treatment option for GPA has not been widely investigated.
A 29-year-old man presented with a 1.0 × 1.0 cm mass without exophthalmos and decreased vision in the left lower eyelid. Computed tomography revealed a mass-like high-density shadow below the left eye with a computed tomography value of 80-108 U.
The laboratory investigations revealed positive cytoplasmic antineutrophil cytoplasmic antibodies (titer = 1:40). Biopsy of the lower left eyelid mass revealed necrosis and granulomatous reaction with a large number of inflammatory cell infiltration. After consultation with the pathology department, the diagnosis was determined as left lower eyelid GPA.
The patient received 9MeV electron beam radiation therapy in the area of the left lower eyelid lesion.
The lesion in the patient was significantly reduced and the symptom relieved obviously. No symptom recurrence or significant toxicity occurred during or after the treatment. The patient remains under routine follow-up.
We present a case of a male patient with GPA located exclusively in the eyelid area, who underwent successful radiotherapy and achieved a complete response. The lesson we learned from this case study is that for GPA patients, when the standard treatment model fails to achieve good results, novel treatments such as radiotherapy should be considered according to the situation.
肉芽肿性多血管炎(GPA)是一种以坏死性肉芽肿性血管炎为特征的慢性系统性血管炎。该疾病主要累及中小血管,主要发生在上呼吸道(鼻和鼻窦)、下呼吸道(肺)和肾脏。眼睑部位发病相对少见。GPA的标准治疗是肾上腺皮质激素与免疫抑制剂联合治疗。放疗作为GPA的一种治疗选择尚未得到广泛研究。
一名29岁男性,左下眼睑出现一个1.0×1.0 cm肿物,无眼球突出及视力下降。计算机断层扫描显示左眼下方有一个肿物样高密度影,计算机断层扫描值为80 - 108 U。
实验室检查显示胞浆型抗中性粒细胞胞浆抗体阳性(滴度 = 1:40)。左下眼睑肿物活检显示坏死及肉芽肿反应,有大量炎性细胞浸润。经与病理科会诊,诊断为左下眼睑GPA。
患者接受了左下眼睑病变区域的9MeV电子束放射治疗。
患者病变明显缩小,症状明显缓解。治疗期间及治疗后均未出现症状复发或明显毒性反应。患者仍在接受常规随访。
我们报告了一例仅累及眼睑部位的男性GPA患者,其接受放疗成功并取得完全缓解。我们从该病例研究中学到的经验是,对于GPA患者,当标准治疗模式未能取得良好效果时,应根据情况考虑放疗等新的治疗方法。