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过犹不及:斯蒂尔病患者的免疫重建炎症综合征

Too much of a good thing: Immune reconstitution inflammatory syndrome in a patient with Still's disease.

作者信息

Batool Nuzhat, Song David, Almas Talal, Alsubai Abdulla K, Thakur Tushar, Ismail Hebatalla, Alsufyani Majid, Hadeed Sebastian, Huang Helen, Kotait Farida, Aldhaheri Khaled Saeed Obaid, Sindi Atif Bakr, Chan Emilie, Salama Carlos

机构信息

Department of Internal Medicine, Icahn School of Medicine at Mount Sinai Elmhurst Hospital, NY, USA.

Royal College of Surgeons in Ireland, Dublin, Ireland.

出版信息

Ann Med Surg (Lond). 2022 Sep 13;82:104590. doi: 10.1016/j.amsu.2022.104590. eCollection 2022 Oct.

DOI:10.1016/j.amsu.2022.104590
PMID:36268454
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9577525/
Abstract

Immune Reconstitution Inflammatory Syndrome (IRIS) is a potential complication when treating non HIV immunosuppressed patients with opportunistic infections. We present a case of a 49-year-old female with Adult-onset Still's disease on prednisone 40 mg daily who came to ED with right leg weakness and intractable headache for one week. She was diagnosed with Cryptococcus meningitis. Patient completed the induction phase of antifungal therapy and the steroids were tapered over four weeks. One month after discharge, a patient was brought in to ED, minimally responsive to verbal stimuli and had new left hemiparesis with persistent right leg weakness was noted on exam. An MRI of the brain was consistent with diffuse leptomeningeal enhancement compatible with meningoencephalitis. LP was notable for elevated opening pressure of 36cmH2O and CSF studies were negative for recurrence of cryptococcal infection. Given the timeline of patients presentation one month after discontinuation of steroids, and workup consistent with sterile meningitis, immune reconstitution inflammatory syndrome was identified as the likely diagnosis. The patient was started on 50 mg of Prednisone daily. Six weeks after presentation, the patient's mental status returned to baseline, left hemiparesis resolved, and right lower extremity strength significantly improved. Clinicians should have a high index of suspicion for CNS IRIS in patients presenting with new neurologic findings in the setting of rapid discontinuation of steroids due to infection. IRIS in HIV patients with cryptococcal meningitis is a well-established entity; the purpose of this case report is to bring attention to similar inflammatory syndrome in non-HIV patients with cryptococcal meningitis.

摘要

免疫重建炎症综合征(IRIS)是治疗非HIV免疫抑制患者机会性感染时的一种潜在并发症。我们报告一例49岁成年女性,患成人斯蒂尔病,每日服用40毫克泼尼松,因右腿无力和顽固性头痛一周前来急诊科就诊。她被诊断为隐球菌性脑膜炎。患者完成了抗真菌治疗的诱导期,类固醇药物在四周内逐渐减量。出院后一个月,患者被送往急诊科,对言语刺激反应微弱,检查发现有新的左侧偏瘫,右腿持续无力。脑部MRI显示弥漫性软脑膜强化,符合脑膜脑炎表现。腰椎穿刺显示初压升高至36cmH2O,脑脊液检查隐球菌感染未复发。鉴于患者在停用类固醇药物一个月后出现症状,且检查结果符合无菌性脑膜炎,免疫重建炎症综合征被确定为可能的诊断。患者开始每日服用50毫克泼尼松。就诊六周后,患者精神状态恢复至基线,左侧偏瘫消失,右下肢力量明显改善。对于因感染而迅速停用类固醇药物后出现新的神经系统症状的患者,临床医生应高度怀疑中枢神经系统IRIS。HIV患者合并隐球菌性脑膜炎时的IRIS是一个已被充分认识的实体;本病例报告的目的是引起对非HIV患者合并隐球菌性脑膜炎时类似炎症综合征的关注。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3c13/9577525/4d2754c25ea0/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3c13/9577525/4d2754c25ea0/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3c13/9577525/4d2754c25ea0/gr1.jpg

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