Department of Rheumatology, Jichi Medical University Saitama Medical Center, Saitama, Japan.
Department of Internal Medicine, Division of Rheumatology, Toho University School of Medicine, Tokyo, Japan.
Mod Rheumatol Case Rep. 2022 Jun 24;6(2):260-265. doi: 10.1093/mrcr/rxab035.
Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown aetiology that is categorised as a non-hereditary disease. Neonatal haemophagocytic lymphohistiocytosis (HLH) is also a rare, but potentially fatal condition. Neonatal HLH is one of the causes of neonatal acute liver failure that often requires urgent liver transplantation. The relationship between AOSD during pregnancy and neonatal HLH currently remains unclear. We encountered a case of AOSD that developed during pregnancy, and an offspring was born with neonatal HLH resulting in severe liver failure. The mother with AOSD only presented with liver dysfunction during pregnancy; however, disease activity was exacerbated after delivery. The maternal clinical course was quite severe and refractory that she required biological therapy in addition to high-dose corticosteroids and immunosuppressants. Additionally, the severe condition of the neonate with HLH and acute liver failure required intensive care with the administration of steroids and intravenous immunoglobulin treatments and ultimately liver transplantation. This is the first case that severe maternal AOSD associated with a neonatal HLH resulted in severe clinical courses. Physicians need to be aware of the risk of a mother with AOSD delivering an offspring with neonatal HLH with potentially acute liver failure.
成人Still 病（AOSD）是一种病因不明的罕见全身性炎症性疾病，归类为非遗传性疾病。新生儿噬血细胞性淋巴组织细胞增生症（HLH）也是一种罕见但潜在致命的疾病。新生儿 HLH 是导致新生儿急性肝功能衰竭的原因之一，通常需要紧急进行肝移植。妊娠期间 AOSD 与新生儿 HLH 之间的关系目前仍不清楚。我们遇到了一例在妊娠期间发生的 AOSD，其新生儿患有新生儿 HLH 导致严重肝功能衰竭。患有 AOSD 的母亲在妊娠期间仅表现为肝功能异常；然而，分娩后疾病活动度加剧。母体的临床病程相当严重且具有抗药性，除了大剂量皮质类固醇和免疫抑制剂外，她还需要接受生物治疗。此外，HLH 和急性肝功能衰竭的新生儿病情严重，需要进行重症监护，给予类固醇和静脉注射免疫球蛋白治疗，最终需要进行肝移植。这是首例严重的母体 AOSD 伴发新生儿 HLH 导致严重临床病程的病例。医生需要意识到患有 AOSD 的母亲可能会生出患有新生儿 HLH 并伴有潜在急性肝功能衰竭的婴儿。
