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[肉芽肿性多血管炎(韦格纳氏):临床病例]

[GRANULOMATOSIS WITH POLYANGIITIS (WEGENER'S): CLINICAL CASE].

作者信息

Zimba E, Olkhova O

机构信息

Danylo Halytsky Lviv National Medical University, Lviv, Ukraine; Andrey Krupynskyy Lviv Institute of Nursing and Laboratory Medicine, Ukraine.

出版信息

Georgian Med News. 2016 May(254):43-7.

PMID:27348166
Abstract

Granulomatosis with polyangiitis (Wegener's disease) - systemic vasculitis, initial manifestations, the clinical picture may be present in a wide variety. This leads to difficulties in establishing a timely diagnosis. The prognosis in untreated generalized granulomatosis with polyangiitis is extremely poor. The present case report illustrates a late diagnosis of granulomatosis with polyangiitis. A 53-year-old woman was diagnosed with granulomatosis with polyangiitis only after ten months of onset of disease. Wrong diagnosis of tuberculosis of ear leads to a lot of delay in the treatment this type of vasculitis. At the time of diagnosis she had generalized form of disease presented with involvement of the eyes, upper and lower respiratory tracts, kidneys, and nervous system. Remission was achieved with methylprednisolone and cyclophosphamide but suffered a relapse shortly afterwards. Further treatment with rituximab achieved a second remission, but the patient continued to suffer from dry conjunctivitis. Symptomatic therapy in this case was ineffective. An effective pathogenic therapy for this condition was instillation of cyclosporine eye drops.

摘要

肉芽肿性多血管炎(韦格纳肉芽肿病)——一种系统性血管炎,其初始表现及临床症状可能多种多样。这导致难以及时做出诊断。未经治疗的全身性肉芽肿性多血管炎预后极差。本病例报告阐述了肉芽肿性多血管炎的延迟诊断情况。一名53岁女性在发病十个月后才被诊断为肉芽肿性多血管炎。耳部结核的误诊导致这类血管炎的治疗延误许久。确诊时,她患有全身性疾病,累及眼睛、上呼吸道和下呼吸道、肾脏及神经系统。甲泼尼龙和环磷酰胺治疗实现了缓解,但不久后病情复发。利妥昔单抗进一步治疗实现了第二次缓解,但患者仍患有干眼症。该病例的对症治疗无效。针对这种情况的有效病因治疗方法是滴注环孢素眼药水。

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