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[鼻窦和眼眶区域慢性双侧坏死性及破坏性肉芽肿性炎症过程作为局限性韦格纳肉芽肿病的表现]

[Chronic bilateral necrotizing and destructive granulomatous inflammatory process in the region of the paranasal sinuses and the orbita as manifestation of localized Wegener's granulomatosis].

作者信息

Keerl Rainer, Löhr Dirk, Kahle Gabriele, Kronsbein Hartmut, Fassbinder Winfried, Draf Wolfgang

机构信息

Klinik für HNO-Heilkunde, Kopf- Hals- und Plastische Gesichtschirurgie, Klinikum Straubing.

出版信息

Med Klin (Munich). 2003 Aug 15;98(8):453-7. doi: 10.1007/s00063-003-1286-5.

Abstract

CASE REPORT

Granulomatous infectious processes have a wide differential diagnosis. This report describes the case of a 73-year-old woman who had gone through an 8-year ordeal involving several paranasal sinus operations, development of chronic facial pain, orbital exenteration of the left eye, and now threatening loss of the remaining right eye on account of progression of the chronic inflammation. Despite repeated histologic examination of ENT material by various pathologic institutes, neither the histology nor laboratory parameters were able to point us in the right direction. In the end, it was the clinical course which led to the diagnosis of Wegener's granulomatosis.

CONCLUSION

In chronic necrotizing granulomatous inflammatory processes in the region of the paranasal sinuses with involvement of the surrounding anatomic structures (orbit, skull base), Wegener's granulomatosis should be included in the differential diagnosis at an early stage, even if the typical signs of vasculitis and the typical antibodies are absent. The clinical course calls for an interdisciplinary treatment approach in conjunction with internists experienced in immunosuppressant therapy.

摘要

病例报告

肉芽肿性感染性疾病的鉴别诊断范围很广。本报告描述了一名73岁女性的病例,她经历了长达8年的磨难,包括多次鼻窦手术、慢性面部疼痛的出现、左眼眼眶内容剜除术,而现在由于慢性炎症的进展,右眼也面临失明的威胁。尽管多家病理机构对耳鼻喉科材料进行了反复的组织学检查,但组织学检查结果和实验室参数均未能为我们指明正确方向。最终,是临床病程引导我们诊断为韦格纳肉芽肿。

结论

在鼻窦区域出现慢性坏死性肉芽肿性炎症并累及周围解剖结构(眼眶、颅底)时,即使没有血管炎的典型体征和典型抗体,早期鉴别诊断也应考虑韦格纳肉芽肿。临床病程需要采用跨学科的治疗方法,并联合有免疫抑制治疗经验的内科医生。

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