A Recurrent Case of Adult-onset Still's Disease with Concurrent Acalculous Cholecystitis and Macrophage Activation Syndrome/Hemophagocytic Lymphohistiocytosis Successfully Treated with Combination Immunosuppressive Therapy

被引：5

作者：

Arai, Yuri ^{[1
]}

Ishikawa, Yuichi ^{[1
]}

Abe, Kazuya ^{[1
]}

Kato, Yuri ^{[2
]}

Abe, Daijiro ^{[3
]}

Fujiwara, Michio ^{[1
]}

Kita, Yasuhiko ^{[1
]}

机构：

[1] Yokohama Rosai Hosp, Dept Rheumatol, Yokohama, Kanagawa, Japan

[2] Yokohama Rosai Hosp, Dept Gastroenterol, Yokohama, Kanagawa, Japan

[3] Yokohama Rosai Hosp, Dept Hematol, Yokohama, Kanagawa, Japan

来源：

INTERNAL MEDICINE | 2021年 / 60卷 / 12期

关键词：

adult-onset Still's disease; macrophage activation syndrome; hemophagocytic lymphohistiocytosis; acalculous cholecystitis; cyclosporine A; CYCLOSPORINE; MANAGEMENT;

D O I：

10.2169/internalmedicine.5781-20

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

We herein report the case of 21-year-old female diagnosed with adult-onset Still's disease (AOSD) three years earlier who presented with fever and right upper abdominal pain. She was diagnosed with acute acalculous cholecystitis (AAC) based on hepatic dysfunction, elevated C-reactive protein, and gallbladder wall thickening on abdominal ultrasound. Based on the presence of pancytopenia, hyperferritinemia, and hemophagocytosis by a bone marrow examination, she was diagnosed with macrophage activation syndrome (MAS)/hemophagocytic lymphohistiocytosis (HLH) which was refractory to glucocorticoid pulse therapy. The combination of intravenous cyclosporine A with glucocorticoids was able to successfully control the disease activity of AOSD-related AAC and MAS/HLH.

引用

页码：1955 / 1961

页数：7

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