著者

Kenji Saito Jumpei Temmoku Yuya Sumichika Shuhei Yoshida Eisuke Takano Shuhei Watanabe Haruki Matsumoto Yuya Fujita Naoki Matsuoka Tomoyuki Asano Shuzo Sato Hiroshi Watanabe Kiyoshi Migita

出版者

The Japanese Society of Internal Medicine

雑誌

Internal Medicine (ISSN:09182918)

巻号頁・発行日

pp.1026-22, (Released:2023-02-22)

参考文献数

32

被引用文献数

1

---

Adult-onset Still's disease (AOSD) is characterized by high spiking fever, evanescent rash, and arthritis. However, AOSD rarely presents with severe acute kidney injury (AKI). We herein present the case of a 56-year-old woman with new-onset AOSD who rapidly developed AKI. A physical examination and laboratory data revealed spiking fever, evanescent rash, thrombocytopenia, hyperferritinemia, and azotemia. The patient was diagnosed with AOSD complicated by AKI and macrophage activation syndrome. Treatment with high-dose steroids, hemodialysis, and plasma exchange successfully resolved her AKI. In this report, we review previously published reports on AOSD accompanied by AKI and discuss this rare complication in AOSD.

著者

Jumpei Temmoku Tomomi Sasajima Takeshi Kuroda Yuya Sumichika Kenji Saito Shuhei Yoshida Haruki Matsumoto Yuya Fujita Naoki Matsuoka Tomoyuki Asano Shuzo Sato Toshiyuki Yamada Yuko Hashimoto Kiyoshi Migita

出版者

Tohoku University Medical Press

雑誌

The Tohoku Journal of Experimental Medicine (ISSN:00408727)

巻号頁・発行日

pp.2023.J003, (Released:2023-01-19)

著者

Kiyoshi Migita Yuya Fujita Tomoyuki Asano Shuzo Sato

出版者

The Japanese Society of Internal Medicine

雑誌

Internal Medicine (ISSN:09182918)

巻号頁・発行日

pp.9279-21, (Released:2022-03-19)

参考文献数

69

被引用文献数

2

---

Autoinflammatory diseases are systemic disorders caused by genetic or acquired abnormalities in certain signaling pathways of the innate immune system. Dysregulated activation of the inflammasome, i.e. molecular platforms responsible for the activation of caspase-1 and production of interleukin-1β, causes autoinflammation. Familial Mediterranean fever (FMF), the most common genetic autoinflammatory disease, is characterized by a periodic fever and serositis. The complex and heterogeneous genetic background of Japanese FMF patients, accompanied by potential overlap with other rheumatic diseases, suggests crosstalk between genetic and environmental factors. Recently, FMF has been recognized as being part of a spectrum of autoinflammatory syndromes named pyrin-associated autoinflammatory diseases. The discovery of a new monogenic autoinflammatory disease, A20 haploinsufficiency, may provide novel insights into early-onset Behçet's-like diseases. In contrast, adult-onset Still's disease and Schnitzler's syndrome are acquired autoinflammatory diseases without a monogenic abnormality. Although the concept of autoinflammatory diseases originally applied to monogenic hereditary recurrent fevers, it has been expanded to include non-genetic complex autoinflammatory diseases. Information concerning monogenic autoinflammatory diseases may prove useful for elucidating the molecular mechanisms underlying non-genetic autoinflammatory diseases.

著者

HIROSHI WATANABE MAKIKO YASHIRO TOMOYUKI ASANO SHUZO SATO ATSUSHI TAKAHASHI KYOKO KATAKURA HIROKO KOBAYASHI HIROMASA OHIRA

出版者

福島医学会

雑誌

FUKUSHIMA JOURNAL OF MEDICAL SCIENCE (ISSN:00162590)

巻号頁・発行日

pp.2014-27, (Released:2015-05-19)

参考文献数

17

被引用文献数

4

---

Stressful life situation can trigger the onset and flare-ups of Behçet’s disease (BD). In addition, the association of systemic sclerosis (SSc) and BD is rare. In this study, we report a patient who had Sjögren’s syndrome as a primary disease and subsequently developed SSc and BD after an earthquake disaster and the death of her father.