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Case Reports
. 2021 Jun 15;60(12):1955-1961.
doi: 10.2169/internalmedicine.5781-20. Epub 2021 Feb 1.

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

Yuri Arai  1 , Yuichi Ishikawa  1 , Kazuya Abe  1 , Yuri Kato  2 , Daijiro Abe  3 , Michio Fujiwara  1 , Yasuhiko Kita  1
Affiliations
Case Reports

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

Yuri Arai et al. Intern Med. .

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.

Keywords: acalculous cholecystitis; adult-onset Still's disease; cyclosporine A; hemophagocytic lymphohistiocytosis; macrophage activation syndrome.

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Conflict of interest statement

The authors state that they have no Conflict of Interest (COI).

Figures

Figure 1.
Figure 1.
Ultrasonography and computed tomography (CT) images on admission. (a) Ultrasonography images of the gallbladder. (b, c) CT images of the gallbladder (arrows), liver, and spleen. (d) CT images of the cervical lymph nodes (arrow). (e) Changes in the gallbladder ultrasonography findings on day 26.
Figure 2.
Figure 2.
Histopathological findings of a bone marrow biopsy sample. (a) Hematopoiesis in the bone marrow is preserved. Histiocytes are shown (red circles) (magnification: 400×). (b) Activated macrophages exhibiting phagocytosis (red arrow) (magnification: 1,000×).
Figure 3.
Figure 3.
Clinical course of the patient. CsA: cyclosporine A, i.v.: intravenous, mPSL: methylprednisolone, PLT: platelet count, p.o.: per os, TAZ/PIPC: tazobactam/piperacillin
See this image and copyright information in PMC

References

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