title: Hemophagocytic syndrome and inflammatory myopathy with abundant macrophages in a patient with adult-onset Still's disease.
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.
authors.affiliation: Department of Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan.
authors.forename: Masataka
authors.initials: M
authors.lastname: Umeda
authors.forename: Tomoki
authors.initials: T
authors.lastname: Origuchi
authors.forename: Keita
authors.initials: K
authors.lastname: Fujikawa
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Koga
authors.forename: Akinari
authors.initials: A
authors.lastname: Mizokami
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Nakashima
authors.forename: Takahisa
authors.initials: T
authors.lastname: Suzuki
authors.forename: Akitomo
authors.initials: A
authors.lastname: Okada
authors.forename: Shin-ya
authors.initials: SY
authors.lastname: Kawashiri
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Ichinose
authors.forename: Mami
authors.initials: M
authors.lastname: Tamai
authors.forename: Satoshi
authors.initials: S
authors.lastname: Yamasaki
authors.forename: Hideki
authors.initials: H
authors.lastname: Nakamura
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kawakami
authors.forename: Katsumi
authors.initials: K
authors.lastname: Eguchi
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D000071457:DEAD Box Protein 58; D053487:DEAD-box RNA Helicases / Q000096:biosynthesis*; D005260:Female; D005334:Fever; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications*; D008264:Macrophages / Q000276:immunology; D009220:Myositis / Q000150:complications* / Q000276:immunology*; D011971:Receptors, Immunologic; D016706:Still's Disease, Adult-Onset / Q000276:immunology* / Q000473:pathology
chemical_list: D011971:Receptors, Immunologic; C452820:RIGI protein, human; D000071457:DEAD Box Protein 58; D053487:DEAD-box RNA Helicases