Wygoda Andrzej, Rutkowski Tomasz, Składowski Krzysztof, Hejduk Beata
I Radiotherapy Clinic, Maria Sklodowska-Curie Memorial Cancer Centre and Institute of Oncology, Branch Gliwice.
Contemp Oncol (Pozn). 2013;17(1):107-11. doi: 10.5114/wo.2013.33785. Epub 2013 Mar 15.
Wegener's granulomatosis is a rare disease (10 per 1 000 000 new cases per year), etiologically connected with necrotizing vasculitis of small to medium-size vessels. The disease occurs predominantly in the upper respiratory tract, lungs and kidneys, but any organ may be affected during the course of the illness. It may be difficult to diagnose, especially when c-ACNA antibodies (serologic symptom of Wegener's granulomatosis) are undetectable and chest X-ray is normal. Early diagnosis is crucial for treatment results. Untreated disease may lead to death. Cyclophosphamide used simultaneously with prednisone is the treatment of choice as the first line procedure. Resistance to standard systemic treatment may be a significant problem. New drugs (rituximab, infliximab) are still under clinical investigation, with promising results. Very limited data concerning effectiveness of radiation therapy exist. We present a report of a female patient with solitary form of Wegener's granulomatosis located in the facial region, who underwent successful radiation therapy with a complete response.
韦格纳肉芽肿是一种罕见疾病（每年每100万人中有10例新发病例），病因与中小血管坏死性血管炎相关。该病主要发生在上呼吸道、肺部和肾脏，但在病程中任何器官都可能受累。诊断可能困难，尤其是当无法检测到抗中性粒细胞胞浆抗体（韦格纳肉芽肿的血清学症状）且胸部X光检查正常时。早期诊断对治疗结果至关重要。未经治疗的疾病可能导致死亡。环磷酰胺与泼尼松同时使用是首选的一线治疗方法。对标准全身治疗的耐药性可能是一个重大问题。新药（利妥昔单抗、英夫利昔单抗）仍在临床研究中，结果令人鼓舞。关于放射治疗有效性的数据非常有限。我们报告了一名患有面部孤立型韦格纳肉芽肿的女性患者，她接受放射治疗后获得成功，完全缓解。
