Amlani Adam, Bromley Amy, Fifi-Mah Aurore
Department of Internal Medicine, University of Calgary, Calgary, AB, Canada.
Department of Pathology, University of Calgary, Calgary, AB, Canada.
Case Rep Rheumatol. 2018 Feb 18;2018:9263537. doi: 10.1155/2018/9263537. eCollection 2018.
A 69-year-old female with antisynthetase syndrome, a history of multiple recurrent infections, and documented previous negative titres for anti-neutrophil cystoplasmic antibody (ANCA) suddenly developed a de novo MPO-ANCA-associated glomerulonephritis three weeks after a fecal microbiota transplantation (FMT) for recurrent infections. Six months following her FMT and less than two weeks following treatment for urosepsis, she developed severe cholestasis, a markedly elevated ferritin and hypertriglyceridemia. An initial liver biopsy was suggestive of drug-induced liver injury and thus she was treated with supportive care. After she failed to improve, a second liver biopsy supported the diagnosis of hemophagocytic lymphohistiocytosis (HLH). This case highlights difficulties surrounding the early diagnosis of HLH and also questions the role of FMT and/or recurrent infections as a trigger for ANCA-associated vasculitis.
一名69岁女性，患有抗合成酶综合征，有多次反复感染史，既往抗中性粒细胞胞浆抗体（ANCA）检测呈阴性，在因反复感染接受粪便微生物群移植（FMT）三周后，突然发生了新发的MPO-ANCA相关性肾小球肾炎。在她接受FMT六个月后，以及在治疗泌尿道感染不到两周后，她出现了严重胆汁淤积、铁蛋白显著升高和高甘油三酯血症。最初的肝脏活检提示药物性肝损伤，因此她接受了支持性治疗。在病情未改善后，第二次肝脏活检支持噬血细胞性淋巴组织细胞增生症（HLH）的诊断。该病例突出了HLH早期诊断的困难，也对FMT和/或反复感染作为ANCA相关性血管炎触发因素的作用提出了质疑。
