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神经结节病:单中心学术医疗系统的纵向经验。

Neurosarcoidosis: Longitudinal experience in a single-center, academic healthcare system.

机构信息

From the Department of Neurology (J.L., M.M.P.S., J. Galli, R.B., L.D.D., J. Klein, J.R., J. Greenlee, S.L.C.), University of Utah; George E. Wahlen Veterans Affairs Medical Center (J.L., M.M.P.S., J. Galli, S.L.C.); Department of Radiology and Imaging Sciences (K.L.S.), and Departments of Internal Medicine and Bioinformatics (J. Kresser), University of Utah, Salt Lake City.

出版信息

Neurol Neuroimmunol Neuroinflamm. 2020 May 13;7(4). doi: 10.1212/NXI.0000000000000743. Print 2020 Jul.

Abstract

OBJECTIVE

To characterize patients with neurosarcoidosis within the University of Utah healthcare system, including demographics, clinical characteristics, treatment, and long-term outcomes.

METHODS

We describe the clinical features and outcomes of patients with neurosarcoidosis within the University of Utah healthcare system (a large referral center for 10% of the continental United States by land mass). Patients were selected who met the following criteria: (1) at least one , 9th revision code 135 or , 10th revision code D86* (sarcoidosis) and (2) at least one outpatient visit with a University of Utah clinician in the Neurology Department within the University of Utah electronic health record.

RESULTS

We identified 56 patients meeting the study criteria. Thirty-five patients (63%) were women, and most patients (84%) were white. Twelve patients (22%) met the criteria for definite neurosarcoidosis, 36 patients (64%) were diagnosed with probable neurosarcoidosis, and 8 patients (14%) were diagnosed with possible neurosarcoidosis. A total of 8 medications were used for the treatment of neurosarcoidosis. Prednisone was the first-line treatment in 51 patients (91%). Infliximab was the most effective therapy, with 87% of patients remaining stable or improving on infliximab. Treatment response for methotrexate and azathioprine was mixed, and mycophenolate mofetil and rituximab were the least effective treatments in this cohort.

CONCLUSIONS

This is a comprehensive characterization of neurosarcoidosis within a single healthcare system at the University of Utah that reports long-term response to treatment and outcomes of patients with neurosarcoidosis. Our results suggest the use of infliximab as a first-line therapy for neurosarcoidosis.

摘要

目的

描述犹他大学医疗系统内的神经结节病患者的特征,包括人口统计学特征、临床特征、治疗方法和长期预后。

方法

我们描述了犹他大学医疗系统(通过陆地面积为美国大陆的 10%提供服务的大型转诊中心)内神经结节病患者的临床特征和结局。选择符合以下标准的患者:(1)至少有一个第 9 版编码 135 或第 10 版编码 D86*(结节病);(2)在犹他大学电子病历中的神经病学系至少有一次与犹他大学临床医生的门诊就诊记录。

结果

我们确定了符合研究标准的 56 名患者。35 名患者(63%)为女性,大多数患者(84%)为白人。12 名患者(22%)符合明确神经结节病的标准,36 名患者(64%)被诊断为可能神经结节病,8 名患者(14%)被诊断为可疑神经结节病。共有 8 种药物用于治疗神经结节病。泼尼松是 51 名患者(91%)的一线治疗药物。英夫利昔单抗是最有效的治疗方法,87%的患者在使用英夫利昔单抗后病情稳定或改善。甲氨蝶呤和硫唑嘌呤的治疗反应不一,而霉酚酸酯和利妥昔单抗在这组患者中疗效最差。

结论

这是对犹他大学单一医疗系统内神经结节病的全面描述,报告了患者对治疗的长期反应和神经结节病的结局。我们的结果表明,英夫利昔单抗可作为神经结节病的一线治疗药物。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/90e8/7238893/dc2162c20c68/NEURIMMINFL2020027672f1.jpg

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