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三例免疫复合物性肾小球肾炎合并特发性颅内高压的病例系列

Case series of idiopathic intracranial hypertension in three patients with immune-complex glomerulonephritis.

机构信息

Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

出版信息

BMC Neurol. 2021 Jul 13;21(1):278. doi: 10.1186/s12883-021-02297-3.

Abstract

BACKGROUND

Idiopathic intracranial hypertension (IIH) is defined by an increased cerebrospinal fluid pressure in the absence of inflammation, structural obstructions, or mass lesions. Although the underlying pathogenesis of IIH is not fully understood, associations with specific risk factors as obesity, obstruction of cerebral venous sinuses, medications, endocrine or systemic conditions and chronic kidney disease have been described. Immune-complex glomerulonephritis as IgA-nephropathy is a frequent cause of chronic kidney failure, which was reported previously in one IIH patient. To date, there is no knowledge about the variable relation of immune-complex nephritis, kidney function and the course of IIH.

CASE PRESENTATION

We report three cases (two females) of concurrent diagnosis of IIH and immune-complex glomerulonephritis. All patients presented with typical IIH symptoms of headache and visual disturbances. Two patients had been diagnosed with IgA-nephropathy only few weeks prior to IIH diagnosis. The third patient had been diagnosed earlier with terminal kidney failure due to a cryoglobulin glomerulonephritis.

CONCLUSION

We propose a possible link between renal deposition of immune-complexes and increased cerebrospinal fluid pressure. Pathophysiological hypotheses and clinical implications are discussed. We recommend clinical awareness and further systematic research to obtain more information on the association of IIH and immune-complex glomerulonephritis.

摘要

背景

特发性颅内高压(IIH)是指在无炎症、结构阻塞或肿块病变的情况下,脑脊液压力升高。尽管 IIH 的潜在发病机制尚未完全阐明,但已描述了与特定危险因素的关联,如肥胖、脑静脉窦阻塞、药物、内分泌或系统性疾病以及慢性肾脏病。免疫复合物性肾小球肾炎如 IgA 肾病是慢性肾衰竭的常见病因,先前曾有一例 IIH 患者报告。迄今为止,尚不清楚免疫复合物性肾炎、肾功能和 IIH 病程之间的可变关系。

病例介绍

我们报告了三例(两例女性)同时诊断为 IIH 和免疫复合物性肾小球肾炎的病例。所有患者均出现 IIH 的典型症状,如头痛和视觉障碍。两名患者在 IIH 诊断前仅数周被诊断为 IgA 肾病。第三名患者因冷球蛋白血症性肾小球肾炎而被诊断为终末期肾衰竭。

结论

我们提出了免疫复合物在肾脏沉积与脑脊液压力升高之间可能存在联系。讨论了病理生理学假说和临床意义。我们建议临床注意并进一步进行系统研究,以获得更多关于 IIH 和免疫复合物性肾小球肾炎之间关联的信息。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5045/8278647/51bebc673ef8/12883_2021_2297_Fig1_HTML.jpg

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