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- Gen Nakamura, Saki Matsuo, Kiyoko Hosaka, Takashi Abe, Kaori Kato, Masahiro Yabe, Shuichi Murakami, Hideki Hashidate, and Daisuke Kondo.
- Department of Nephrology and Rheumatology, Niigata City General Hospital, Japan.
- Intern. Med. 2023 Dec 15; 62 (24): 369136973691-3697.
AbstractInfection can induce hemophagocytic lymphohistiocytosis (HLH) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We herein report a 52-year-old man who had HLH and AAV simultaneously, possibly caused by Enterococcus faecalis infective endocarditis. The HLH diagnosis was based on the HLH-2004 criteria. AAV was diagnosed based on a positive result for proteinase-3 ANCA and necrotizing vasculitis of the small vessels on a skin biopsy. He eventually died and was sent for autopsy after combination treatment of valve replacement, antibiotics, and immunosuppressants, including corticosteroids. This case involved a challenging diagnosis and treatment of HLH with various complications in an adult.
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