Hemophagocytic Syndrome and Inflammatory Myopathy with Abundant Macrophages in a Patient with Adult-onset Still's Disease

Masataka Umeda; Tomoki Origuchi; Keita Fujikawa; Tomohiro Koga; Akinari Mizokami; Yoshikazu Nakashima; Takahisa Suzuki; Akitomo Okada; Shin-ya Kawashiri; Kunihiro Ichinose; Mami Tamai; Satoshi Yamasaki; Hideki Nakamura; Atsushi Kawakami; Katsumi Eguchi

doi:10.2169/internalmedicine.53.1081

CASE REPORTS

Hemophagocytic Syndrome and Inflammatory Myopathy with Abundant Macrophages in a Patient with Adult-onset Still's Disease

Masataka Umeda, Tomoki Origuchi, Keita Fujikawa, Tomohiro Koga, Akinari Mizokami, Yoshikazu Nakashima, Takahisa Suzuki, Akitomo Okada, Shin-ya Kawashiri, Kunihiro Ichinose, Mami Tamai, Satoshi Yamasaki, Hideki Nakamura, Atsushi Kawakami, Katsumi Eguchi

Author information

Masataka Umeda
Department of Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Department of Rheumatology, Sasebo Chuo Hospital, Japan
Tomoki Origuchi
Department of Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Department of Rehabilitation Sciences, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Keita Fujikawa
Department of Rheumatology, Isahaya Health Insurance General Hospital, Japan
Tomohiro Koga
Department of Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Akinari Mizokami
Department of Internal Medicine, Nagasaki Munincipal Hospital, Japan
Yoshikazu Nakashima
Department of Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Takahisa Suzuki
Department of Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Akitomo Okada
Department of Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Shin-ya Kawashiri
Department of Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Kunihiro Ichinose
Department of Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Mami Tamai
Department of Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Satoshi Yamasaki
Department of Rheumatology, Hiroshima University Hospital, Japan
Hideki Nakamura
Department of Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Atsushi Kawakami
Department of Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Katsumi Eguchi
Department of Internal Medicine, Sasebo Munincipal Hospital, Japan

Keywords: inflammatory myopathy with abundant macrophages, hemophagocytic syndrome, adult-onset Still's disease

JOURNAL OPEN ACCESS

2014 Volume 53 Issue 20 Pages 2385-2389

DOI https://doi.org/10.2169/internalmedicine.53.1081

Details

Abstract

We herein describe a 71-year-old woman with adult-onset Still's disease (AOSD) who developed fever, myalgia, and pancytopenia. The bone marrow aspiration and muscle biopsy revealed hemophagocytic syndrome (HPS) and inflammatory myopathy with abundant macrophages (IMAM). Immunostained specimens were positive for expression of retinoic acid-inducible gene-I (RIG-I), which recognizes viral RNA in infiltrated mononuclear cells as well as muscle tissues. These findings suggest that RIG-I may be involved in induction of HPS and IMAM in AOSD.

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