Human immunodeficiency virus-associated vacuolar encephalomyelopathy with granulomatous-lymphocytic interstitial lung disease improved after antiretroviral therapy: a case report
Human immunodeficiency virus-associated vacuolar encephalomyelopathy with granulomatous-lymphocytic interstitial lung disease improved after antiretroviral therapy: a case report
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DOI：
10.1186/s12981-020-00295-y
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发表时间：
2020-07-09
影响因子：
2.2
通讯作者：
Mukae, Hiroshi
中科院分区：
文献类型：
--
作者：
Akagi, Kazumasa;Yamamoto, Kazuko;Mukae, Hiroshi
背景：在抗逆转录病毒治疗（ART）之前的时代，嗜酸性脑脊髓病是人类免疫缺陷病毒（HIV）-1感染终末期的一种主要神经系统疾病，最近被忽视诊断。肉芽肿性淋巴细胞间质性肺病（GLILD）被经典地确定为常见的可变免疫缺陷的非感染性并发症;然而，它现在被认为是其他免疫缺陷疾病。在这里，我们报告的第一例GLILD伴随空泡性脑脊髓病在新诊断的HIV感染man. Case介绍一个40岁的日本男子提出慢性干咳和进行性截瘫。放射学检查显示双肺弥漫性肺异常、脊髓局灶性脱髓鞘病变和脑白色病变。根据支气管肺泡灌洗中检测到的显著淋巴细胞增多症和经支气管活检证实的T细胞间质性肺病伴肉芽肿，他被诊断为GLILD。微生物检查未发现病原体。该患者还被诊断为艾滋病毒相关空泡性脑脊髓病的基础上升高的艾滋病毒载量在脑脊液。开始ART后，脑病变和截瘫明显改善，肺间质异常和咳嗽消失。结论本报告强调，即使在后ART时代的发达国家与先进的医疗服务，HIV相关空泡性脑脊髓病应考虑在进行性神经系统疾病的鉴别诊断在第一次访问。此外，GLILD可能代表可通过ART治疗的HIV相关肺部表现。
Background Vacuolar encephalomyelopathy, a disregarded diagnosis lately, was a major neurological disease in the terminal stages of human immunodeficiency virus (HIV)-1 infection in the pre-antiretroviral therapy (ART) era. Granulomatous-lymphocytic interstitial lung disease (GLILD) was classically identified as a non-infectious complication of common variable immunodeficiency; however, it is now being recognized in other immunodeficiency disorders. Here, we report the first case of GLILD accompanied by vacuolar encephalomyelopathy in a newly diagnosed HIV-infected man. Case presentation A 40-year-old Japanese man presented with chronic dry cough and progressing paraplegia. Radiological examination revealed diffuse pulmonary abnormalities in bilateral lungs, focal demyelinating lesions of the spinal cord, and white matter lesions in the brain. He was diagnosed with GLILD based on marked lymphocytosis detecting in bronchoalveolar lavage, and transbronchial-biopsy proven T-cellular interstitial lung disease with granulomas. Microbiological examinations did not reveal an etiologic agent. The patient was also diagnosed with HIV-associated vacuolar encephalomyelopathy on the basis of an elevated HIV viral load in cerebrospinal fluid. After initiating ART, the brain lesions and paraplegia improved significantly, and interstitial abnormalities of the lungs and cough disappeared. Conclusion This report highlights that even in the post-ART era in developed countries with advanced healthcare services, HIV-associated vacuolar encephalomyelopathy should be considered in the differential diagnosis of a progressive neurological disorder during the first visit. Furthermore, GLILD may represent an HIV-associated pulmonary manifestation that can be treated by ART.