ANCA Vasculitis and Hemophagocytic Lymphohistiocytosis following a Fecal Microbiota Transplant

Abstract

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 Clostridium difficile 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.

Hemophagocytic lymphohistiocytosis as a presentation of inflammatory bowel disease

“…IBD can be developed due to an excessive bacterial translocation into the bowel wall or dysregulated bacterial control in genetically susceptible hosts 8 . According to the literature, no studies have characterized intestinal microbiota in HLH patients; however, the development of HLH after a fecal microbiota transplant has been reported 9 . Multiple studies have observed the interaction between immunological diseases and disorders in the intestinal microbiota 10 .…”

Hemophagocytic Lymphohistiocytosis and Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis Possibly Caused by <i>Enterococcus faecalis</i> Infective Endocarditis

“…Regarding vasculitis, MAS has been reported in 1.11% of Kawasaki disease cases (22). Some case reports have described patients with polyarteritis nodosa (23,24) or AAV (25)(26)(27) who developed MAS. However, in these reported cases, complicated infections that may trigger the onset of MAS have been identified.…”