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蛛网膜下腔出血揭示的结节性多动脉炎合并原发性混合性冷球蛋白血症

Polyarteritis nodosa associated with essential mixed cryglobulinemia revealed by subarachnoid hemorrhage.

作者信息

Taieb Guillaume, Renard Dimitri, Briere Mathieu, Conte Benjamin, Perrigault Pierre-Francois, Rigau Valerie, Bonafe Alain, Labauge Pierre

机构信息

Department of Neurology, Nîmes University Medical Center, Caremeau Hospital, France.

出版信息

Intern Med. 2011;50(23):2905-9. doi: 10.2169/internalmedicine.50.6153. Epub 2011 Dec 1.

Abstract

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis mediated by intravascular deposition of immune complexes. We report a 55-year-old man with PAN revealed by CNS vasculitis with subarachnoid hemorrhage and brainstem vasogenic oedema, associated with essential mixed cryoglobulinemia. In spite of aggressive treatment (steroids and cyclophosphamide), the patient became tetraplegic. A pathophysiological relationship between cryoglobulin and PAN can be suspected. Mixed cryoglobulinemia may trigger vasculitis with PAN features, possibly explaining the lack of treatment response.

摘要

结节性多动脉炎(PAN)是一种由免疫复合物血管内沉积介导的系统性坏死性血管炎。我们报告了一名55岁的男性,患有PAN,表现为中枢神经系统血管炎伴蛛网膜下腔出血和脑干血管源性水肿,与原发性混合性冷球蛋白血症相关。尽管进行了积极治疗(使用类固醇和环磷酰胺),患者仍出现四肢瘫痪。可以怀疑冷球蛋白与PAN之间存在病理生理关系。混合性冷球蛋白血症可能引发具有PAN特征的血管炎,这可能解释了治疗反应不佳的原因。

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