Abstract：
BACKGROUND: The knowledge of central nervous system (CNS) histoplasmosis is limited to case reports and series.
OBJECTIVE: Our objective was to synthesise clinical, radiological and laboratory characteristics of CNS histoplasmosis to improve our understanding of this rare disease.
METHODS: We performed a systematic review using Pubmed/MEDLINE, Embase and LILACS databases accessed on March 2023 without publication date restrictions. Inclusion criteria comprised: (1) histopathological, microbiological, antigen or serological evidence of histoplasmosis; (2) CNS involvement based on cerebrospinal fluid pleocytosis or neuroimaging abnormalities. We classified the certainty of the diagnosis in proven (CNS microbiological and histopathological confirmation), probable (CNS serological and antigen confirmation) or possible (non-CNS evidence of histoplasmosis). Metaproportion was used to provide a summary measure with 95% confidence intervals for the clinical, radiological and laboratory characteristics. Chi-squared test was used to compare mortality between pairs of antifungal drugs.
RESULTS: We included 108 studies with 298 patients. The median age was 31 years, predominantly male, and only 23% were immunocompromised (134/276, 95%CI: 3-71), mainly due to HIV infection. The most common CNS symptom was headache (130/236, 55%, 95%CI: 49-61), with a duration predominantly of weeks or months. Radiological presentation included histoplasmoma (79/185, 34%, 95%CI: 14-61), meningitis (29/185, 14%, 95%CI: 7-25), hydrocephalus (41/185, 37%, 95%CI: 7-83) and vasculitis (18/185, 6%, 95%CI: 1-22). There were 124 proven cases, 112 probable cases and 40 possible cases. The majority of patients presented positive results in CNS pathology (90%), serology (CSF: 72%; serum: 70%) or CSF antigen (74%). Mortality was high (28%, 56/198), but lower in patients who used liposomal amphotericin B and itraconazole. Relapse occurred in 13% (23/179), particularly in HIV patients, but less frequently in patients who used itraconazole.
CONCLUSIONS: Central nervous system histoplasmosis usually presents subacute-to-chronic symptoms in young adults. Neuroimaging patterns included not only focal lesions but also hydrocephalus, meningitis and vasculitis. Positive results were commonly found in CSF antigen and serology. Mortality was high, and treatment with liposomal amphotericin B followed by itraconazole may decrease mortality.
OBJECTIVE: Our objective was to synthesise clinical, radiological and laboratory characteristics of CNS histoplasmosis to improve our understanding of this rare disease.
METHODS: We performed a systematic review using Pubmed/MEDLINE, Embase and LILACS databases accessed on March 2023 without publication date restrictions. Inclusion criteria comprised: (1) histopathological, microbiological, antigen or serological evidence of histoplasmosis; (2) CNS involvement based on cerebrospinal fluid pleocytosis or neuroimaging abnormalities. We classified the certainty of the diagnosis in proven (CNS microbiological and histopathological confirmation), probable (CNS serological and antigen confirmation) or possible (non-CNS evidence of histoplasmosis). Metaproportion was used to provide a summary measure with 95% confidence intervals for the clinical, radiological and laboratory characteristics. Chi-squared test was used to compare mortality between pairs of antifungal drugs.
RESULTS: We included 108 studies with 298 patients. The median age was 31 years, predominantly male, and only 23% were immunocompromised (134/276, 95%CI: 3-71), mainly due to HIV infection. The most common CNS symptom was headache (130/236, 55%, 95%CI: 49-61), with a duration predominantly of weeks or months. Radiological presentation included histoplasmoma (79/185, 34%, 95%CI: 14-61), meningitis (29/185, 14%, 95%CI: 7-25), hydrocephalus (41/185, 37%, 95%CI: 7-83) and vasculitis (18/185, 6%, 95%CI: 1-22). There were 124 proven cases, 112 probable cases and 40 possible cases. The majority of patients presented positive results in CNS pathology (90%), serology (CSF: 72%; serum: 70%) or CSF antigen (74%). Mortality was high (28%, 56/198), but lower in patients who used liposomal amphotericin B and itraconazole. Relapse occurred in 13% (23/179), particularly in HIV patients, but less frequently in patients who used itraconazole.
CONCLUSIONS: Central nervous system histoplasmosis usually presents subacute-to-chronic symptoms in young adults. Neuroimaging patterns included not only focal lesions but also hydrocephalus, meningitis and vasculitis. Positive results were commonly found in CSF antigen and serology. Mortality was high, and treatment with liposomal amphotericin B followed by itraconazole may decrease mortality.
摘要：
背景：中枢神经系统（CNS）组织胞浆菌病的知识仅限于病例报告和系列。
目的：我们的目的是综合临床，中枢神经系统组织胞浆菌病的放射学和实验室特征，以提高我们对这种罕见疾病的认识。
方法：我们使用Pubmed/MEDLINE进行了系统综述，Embase和LILACS数据库于2023年3月访问，没有发布日期限制。纳入标准包括：（1）组织病理学，微生物,组织胞浆菌病的抗原或血清学证据；（2）基于脑脊液细胞增多或神经影像学异常的中枢神经系统受累。我们将诊断的确定性分类为已证实（中枢神经系统微生物学和组织病理学确认），可能（CNS血清学和抗原确认）或可能（组织胞浆菌病的非CNS证据）。元比例用于为临床提供具有95%置信区间的汇总测量，放射学和实验室特性。卡方检验用于比较抗真菌药物对之间的死亡率。
结果：我们纳入了108项研究，共298例患者。中位年龄为31岁,主要是男性,只有23%的人免疫受损(134/276，95CI:3-71)，主要是由于艾滋病毒感染。最常见的中枢神经系统症状是头痛(130/236，55%，95CI:49-61)，持续时间主要为数周或数月。放射学表现包括组织胞浆瘤(79/185，34%，95CI：14-61），脑膜炎（29/185，14%，95CI：7-25），脑积水（41/185，37%，95CI：7-83）和血管炎（18/185，6%，95CI：1-22）。有124例证实的病例，112例可能病例和40例可能病例。大多数患者的中枢神经系统病理学结果呈阳性（90％），血清学（CSF：72％；血清：70％）或CSF抗原（74％）。死亡率很高（28%，56/198)，但使用脂质体两性霉素B和伊曲康唑的患者较低。复发发生率为13%（23/179），特别是在艾滋病毒患者中，但在使用伊曲康唑的患者中频率较低。
结论：中枢神经系统组织胞浆菌病通常在年轻人中呈现亚急性至慢性症状。神经成像模式不仅包括局灶性病变，还包括脑积水，脑膜炎和血管炎.阳性结果常见于脑脊液抗原和血清学。死亡率很高，用两性霉素B脂质体联合伊曲康唑治疗可降低死亡率.
目的：我们的目的是综合临床，中枢神经系统组织胞浆菌病的放射学和实验室特征，以提高我们对这种罕见疾病的认识。
方法：我们使用Pubmed/MEDLINE进行了系统综述，Embase和LILACS数据库于2023年3月访问，没有发布日期限制。纳入标准包括：（1）组织病理学，微生物,组织胞浆菌病的抗原或血清学证据；（2）基于脑脊液细胞增多或神经影像学异常的中枢神经系统受累。我们将诊断的确定性分类为已证实（中枢神经系统微生物学和组织病理学确认），可能（CNS血清学和抗原确认）或可能（组织胞浆菌病的非CNS证据）。元比例用于为临床提供具有95%置信区间的汇总测量，放射学和实验室特性。卡方检验用于比较抗真菌药物对之间的死亡率。
结果：我们纳入了108项研究，共298例患者。中位年龄为31岁,主要是男性,只有23%的人免疫受损(134/276，95CI:3-71)，主要是由于艾滋病毒感染。最常见的中枢神经系统症状是头痛(130/236，55%，95CI:49-61)，持续时间主要为数周或数月。放射学表现包括组织胞浆瘤(79/185，34%，95CI：14-61），脑膜炎（29/185，14%，95CI：7-25），脑积水（41/185，37%，95CI：7-83）和血管炎（18/185，6%，95CI：1-22）。有124例证实的病例，112例可能病例和40例可能病例。大多数患者的中枢神经系统病理学结果呈阳性（90％），血清学（CSF：72％；血清：70％）或CSF抗原（74％）。死亡率很高（28%，56/198)，但使用脂质体两性霉素B和伊曲康唑的患者较低。复发发生率为13%（23/179），特别是在艾滋病毒患者中，但在使用伊曲康唑的患者中频率较低。
结论：中枢神经系统组织胞浆菌病通常在年轻人中呈现亚急性至慢性症状。神经成像模式不仅包括局灶性病变，还包括脑积水，脑膜炎和血管炎.阳性结果常见于脑脊液抗原和血清学。死亡率很高，用两性霉素B脂质体联合伊曲康唑治疗可降低死亡率.
