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 |
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Authors: | Yuri Arai Yuichi Ishikawa Kazuya Abe Yuri Kato Daijiro Abe Michio Fujiwara Yasuhiko Kita |
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Institution: | 1.Department of Rheumatology, Yokohama Rosai Hospital, Japan; 2.Department of Gastroenterology, Yokohama Rosai Hospital, Japan; 3.Department of Hematology, Yokohama Rosai Hospital, Japan |
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Abstract: | 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. |
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Keywords: | adult-onset Still''s disease macrophage activation syndrome hemophagocytic lymphohistiocytosis acalculous cholecystitis cyclosporine A |
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