a Department of Neurology and Rehabilitation Medicine , University of Cincinnati , Cincinnati , OH , USA.
b Department of Neurology , Medical College of Wisconsin , Milwaukee , WI , USA.
Int J Neurosci. 2019 Jun;129(6):619-622. doi: 10.1080/00207454.2018.1544130. Epub 2018 Nov 28.
CNS involvement in sarcoidosis is seen in 5-10% of cases. Long term treatment involves steroids and other immunomodulatory agents, including infliximab. Chronic immunosuppression can result in increased patient susceptibility to opportunistic infections. We present a case of fatal aspergillosis in a patient with neurosarcoidosis treated with infliximab.
A 55-year-old woman with neurosarcoidosis on infliximab (started 4 months prior) and dexamethasone, presented with progressive cognitive decline. Exam revealed impaired attention and disorientation with preserved language. Brain MRI showed multiple, bilateral cortical and subcortical ring-enhancing lesions. We held immunosuppression due to suspicion of infection; empiric Amphotericin B was given early in the hospital course. The patient rapidly deteriorated from a neurological and respiratory standpoint, requiring intubation. CSF analysis showed elevated protein of 511 and normal glucose of 104 (67% serum), with lymphocytic pleocytosis (25 cells, 96% lymphocytes). Systemic and CNS microbiological studies were negative. On hospital day 13, bronchial fluid grew Aspergillus fumigatus, prompting a switch to voriconazole. Despite early empiric antifungal treatment, she died from respiratory failure; autopsy revealed systemic and CNS aspergillosis with multiple brain abscesses.
This case represents an example of a fatal complication of infliximab therapy, which was recently shown to be effective in neurosarcoidosis in one study. It also serves to highlight the challenges faced in diagnosing ring-enhancing lesions, especially in patients with pre-existing brain disorders. Finally, it highlights the difficulty in treating invasive aspergillosis. Further studies are needed to identify risks associated with infliximab therapy and potential early interventions to improve outcomes.
在 5-10%的病例中,中枢神经系统(CNS)受累于结节病。长期治疗涉及类固醇和其他免疫调节剂,包括英夫利昔单抗。慢性免疫抑制会导致患者易患机会性感染。我们报告了一例接受英夫利昔单抗治疗的神经结节病患者致命性曲霉菌病的病例。
一名 55 岁女性,患有神经结节病,正在接受英夫利昔单抗(4 个月前开始)和地塞米松治疗,出现进行性认知能力下降。检查发现注意力和定向力受损,语言保留。脑部 MRI 显示多个双侧皮质和皮质下环形增强病变。由于怀疑感染,我们抑制了免疫;在住院期间早期给予经验性两性霉素 B。患者从神经和呼吸方面迅速恶化,需要插管。CSF 分析显示蛋白质升高至 511,葡萄糖正常为 104(67%血清),淋巴细胞增多(25 个细胞,96%淋巴细胞)。全身和中枢神经系统微生物学研究均为阴性。在住院第 13 天,支气管液中培养出烟曲霉菌,促使改用伏立康唑。尽管早期进行了经验性抗真菌治疗,但她仍因呼吸衰竭死亡;尸检显示系统性和中枢神经系统曲霉病伴多个脑脓肿。
该病例代表了英夫利昔单抗治疗的致命并发症的一个例子,最近一项研究表明其在神经结节病中有效。它还突出了诊断环形增强病变的挑战,尤其是在有先前存在的脑部疾病的患者中。最后,它强调了治疗侵袭性曲霉菌病的困难。需要进一步研究来确定与英夫利昔单抗治疗相关的风险以及改善结果的潜在早期干预措施。