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.

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.

• Authors: Yuri Arai, Yuichi Ishikawa, Kazuya Abe, Yuri Kato, Daijiro Abe, Michio Fujiwara, Yasuhiko Kita
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 60 Issue 12 Pg. 1955-1961 (Jun 15 2021) ISSN: 1349-7235 [Electronic] Japan
• PMID: 33518559 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunosuppressive Agents

Topics

• Acalculous Cholecystitis (complications, drug therapy)
• Adult
• Female
• Humans
• Immunosuppressive Agents (therapeutic use)
• Lymphohistiocytosis, Hemophagocytic (complications, diagnosis, drug therapy)
• Macrophage Activation Syndrome (diagnosis, drug therapy)
• Still's Disease, Adult-Onset (complications, diagnosis, drug therapy)
• Young Adult