Department of Paediatric Gastroenterology and Nutrition, Royal Hospital for Sick Children, Edinburgh, Uk.
Child Life and Health, College of Medicine and Veterinary Medicine, University of Edinburgh, Edinburgh, UK.
J Crohns Colitis. 2021 Apr 6;15(4):687-691. doi: 10.1093/ecco-jcc/jjaa201.
Paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 [PIMS-TS] is a newly described condition. It has a spectrum of presentations proposed to occur as part of a post-infectious immune response. We report the first case of PIMS-TS in a child on established anti-tumour necrosis factor alpha [anti-TNFα] therapy; a 10 year-old girl with ulcerative colitis treated with infliximab. The patient had 6 weeks of daily fever with mucocutaneous, gastrointestinal, renal, and haematological involvement. Biomarkers of hyperinflammation were present including: hyperferritinaemia [up to 691 µ/L; normal 15-80 µg/L], C-reactive protein [CRP] [ >100mg/L for >10 days, normal 0-5 mg/L], erythrocyte sedimentation rate [ESR] consistently >100mm/h [normal 0-15 mm/h], raised white cell count with neutrophilia, elevated D-dimer and lactate dehydrogenase [LDH], anaemia and Mott cells on bone marrow analysis. Extensive investigations for alternative diagnoses for pyrexia of unknown origin [PUO] were negative. The condition was refractory to treatment with intravenous immunoglobulin [IVIG] but improved within 24 h of high-dose methylprednisolone. Infliximab treatment followed and the patient has remained well at follow-up. Polymerase chain reaction [PCR] and serology for SARS-CoV-2 were negative. Current series report such negative findings in up to half of cases. The patient experienced a milder clinical phenotype without cardiac involvement, shock, or organ failure. Accepting the wide spectrum of PIMS-TS presentations, it is possible that previous anti-TNFα therapy may have attenuated the disease course. Given the uncertainty around therapeutic strategies for PIMS-TS, this case supports the need for further investigation into continuing infliximab as a treatment option for the condition.
儿童炎症性多系统综合征与 SARS-CoV-2 相关(PIMS-TS)是一种新描述的病症。它具有一系列表现,据推测是作为感染后免疫反应的一部分发生的。我们报告了首例在接受抗肿瘤坏死因子-α(anti-TNFα)治疗的儿童中出现的 PIMS-TS 病例;这是一名 10 岁患有溃疡性结肠炎的女孩,接受英夫利昔单抗治疗。该患者有 6 周的每日发热,伴有黏膜皮肤、胃肠道、肾脏和血液系统受累。存在炎症反应的生物标志物升高,包括:高血铁蛋白血症(高达 691µg/L;正常 15-80µg/L)、C 反应蛋白(CRP)[>100mg/L 持续 10 天以上,正常 0-5mg/L]、红细胞沉降率(ESR)持续>100mm/h(正常 0-15mm/h)、白细胞计数升高伴中性粒细胞增多、D-二聚体和乳酸脱氢酶(LDH)升高、骨髓分析有贫血和 Mott 细胞。对发热原因不明(PUO)的其他诊断进行了广泛的检查,结果均为阴性。静脉注射免疫球蛋白(IVIG)治疗无效,但大剂量甲基强的松龙治疗后 24 小时内病情改善。随后继续使用英夫利昔单抗治疗,患者在随访中情况良好。PCR 和 SARS-CoV-2 血清学检查均为阴性。目前的系列报告中,多达一半的病例有这样的阴性发现。该患者的临床表型较轻,无心脏受累、休克或器官衰竭。鉴于 PIMS-TS 的临床表现广泛,之前的抗 TNFα 治疗可能减轻了疾病的进程。鉴于 PIMS-TS 的治疗策略存在不确定性,该病例支持进一步研究继续使用英夫利昔单抗作为该疾病的治疗选择。