著者

Ayako Saito Yoichi Takeuchi Saeko Kagaya Yoshie Ojima Hirotaka Fukami Hiroyuki Sato Ken Matsuda Tasuku Nagasawa

出版者

東北ジャーナル刊行会

雑誌

The Tohoku Journal of Experimental Medicine (ISSN:00408727)

巻号頁・発行日

vol.242, no.1, pp.53-62, 2017 (Released:2017-05-24)

参考文献数

36

被引用文献数

7

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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is systemic vascular inflammation. Microscopic polyangiitis (MPA) is a major type of AAV in Japan. MPA often affects the kidneys and lungs, leading to death if untreated. Induction therapy (i.e., initial treatment) for MPA has not been optimized, although methylprednisolone and cyclophosphamide are commonly used. Recently, rituximab (RTX) (a monoclonal antibody against the protein CD20) has also been used to treat refractory AAV. RTX at 375 mg/m2/week for 4 weeks (i.e., the conventional lymphoma dosing schedule) is used, but the optimal dosing schedule is controversial. Indeed, a single-dose of RTX successfully controlled nephrotic syndrome. However, to date, the effectiveness of a single RTX dose in treating MPA has not been fully investigated in Japan. This was a retrospective observational study. Six newly diagnosed patients with MPA were initially treated with methylprednisolone and a single dose of RTX (375 mg/m2). We investigated the patients’ clinical features, as well as the efficacy and safety of RTX treatment. All patients attained remission on a tapered prednisolone dose of < 10 mg/day during the first 12 months. One patient relapsed after 12 months whereas another required hospitalization owing to infective spondyloarthritis. Adverse reactions to RTX infusion and late-onset neutropenia were not observed. Therefore, a single-dose treatment with RTX induced remission with few complications, and allowed tapering the prednisolone treatment. We conclude that a single dose of RTX is a promising induction therapy for MPA, reducing the cost associated with multiple doses.

著者

Yuki Chiba Kei Takahashi Rui Makino Mai Yoshida Yuji Oe Tasuku Nagasawa Hiroshi Sato Mariko Miyazaki Koji Okamoto

出版者

The Japanese Society of Internal Medicine

雑誌

Internal Medicine (ISSN:09182918)

巻号頁・発行日

pp.8385-21, (Released:2021-12-28)

参考文献数

21

被引用文献数

1

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We herein report a case of crescentic glomerulonephritis associated with infective endocarditis (IE). A 61-year-old-woman presented with a fever and renal dysfunction and was diagnosed with IE. The patient was positive for PR3-ANCA and anti-GBM antibodies. Renal biopsy findings showed crescentic glomerulonephritis with isolated deposition of C3c, a serum conversion product of complement C3. Given these clinical findings, the patient was diagnosed with IE-associated glomerulonephritis. Antibiotic therapy was continued without immunosuppressive agents. After the initiation of the antibiotics, the fever resolved, and the renal function gradually recovered. This case highlights the notion that laboratory findings should be carefully evaluated with reference to other findings.

著者

Yuki Chiba Tasuku Nagasawa

出版者

The Japanese Society of Internal Medicine

雑誌

Internal Medicine (ISSN:09182918)

巻号頁・発行日

vol.60, no.24, pp.4001-4002, 2021-12-15 (Released:2021-12-15)

参考文献数

1

著者

Yoshie Ojima Kinya Sawada Hiroshi Fujii Tsuyoshi Shirai Ayako Saito Saeko Kagaya Satoshi Aoki Yoichi Takeuchi Tomonori Ishii Tasuku Nagasawa

出版者

The Japanese Society of Internal Medicine

雑誌

Internal Medicine (ISSN:09182918)

巻号頁・発行日

vol.57, no.9, pp.1301-1308, 2018-05-01 (Released:2018-05-01)

参考文献数

47

被引用文献数

7

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A previously healthy 58-year-old man was admitted for muscle pain and weakness [manual muscle testing (MMT) of 4/4 for upper and lower limbs]. We detected elevated levels of inflammatory makers and PR3-anti-neutrophil cytoplasmic antibody (ANCA). Subsequently, the muscle weakness rapidly progressed to an MMT of 2 for all limbs. Magnetic resonance imaging indicated muscle edema, and the creatine kinase (CK) level increased to 29,998 U/L. Methylprednisolone (mPSL) and cyclophosphamide pulse therapy improved the patient symptoms. MMT recovered to 4 for all limbs. A muscle biopsy showed degenerated muscle fibers surrounded by neutrophil-predominant infiltration. In addition, lamina elastic breakdown and fibrinoid necrosis of arterioles were observed. A final diagnosis of microscopic polyangiitis (MPA) limited to the muscles was made.

著者

Yusuke Kobari Tasuku Nagasawa

出版者

The Japanese Society of Internal Medicine

雑誌

Internal Medicine (ISSN:09182918)

巻号頁・発行日

vol.56, no.24, pp.3365-3367, 2017-12-15 (Released:2017-12-15)

参考文献数

4

被引用文献数

2 7

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A previously healthy 77-year-old Japanese man presented with a 2-week history of daily fevers peaking at 38°C, chills, hearing loss, and almost 10 kg of unintentional weight loss over 2 months. Pure tone audiometry showed mixed conductive-sensorineural hearing loss: right, 63.6 dB, left, 80.0 dB. Blood tests after admission showed a high myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) level (>300 U/mL), so we suspected ANCA-related vasculitis. The Japanese Otorhinolaryngology Society has recently been advocating the concept of otitis media with ANCA-associated vasculitis (OMAAV). Our case met the criteria proposed, leading to our diagnosis.

著者

Satoshi Kumakura Takashi Nakamichi Nonoka Suzuki Shu Yamakage Ayako Ishikawa Emi Fujikura Satoko Sato Takeshi Aoki Hiroaki Musha Katsuko Kikuchi Tasuku Nagasawa Hiroshi Sato Sadayoshi Ito Mariko Miyazaki

出版者

The Japanese Society of Internal Medicine

雑誌

Internal Medicine (ISSN:09182918)

巻号頁・発行日

pp.2378-18, (Released:2019-02-01)

参考文献数

22

被引用文献数

4

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A 66-year-old man was admitted to our hospital because of multiple refractory skin ulcers. Based on his severe systemic arterial calcification and severe calcium-phosphate imbalance due to severe kidney dysfunction, we initially considered calciphylaxis. However, a skin biopsy provided a diagnosis of cholesterol crystal embolization. Although we initiated hemodialysis, steroid treatment, and low-density lipoprotein-cholesterol apheresis, he died of multiple intestinal perforation. An autopsy showed cholesterol crystals occluding multiple organ arterioles. This case suggests that skin ulcers in patients with chronic kidney disease may be an important diagnostic hallmark and may be associated with several serious diseases.

著者

Saori Yamamoto Tasuku Nagasawa Koichiro Sugimura Atuhiro Kanno Shunsuke Tatebe Tatsuo Aoki Haruka Sato Katsuya Kozu Ryo Konno Kotaro Nochioka Kimio Sato Hiroaki Shimokawa

出版者

The Japanese Society of Internal Medicine

雑誌

Internal Medicine (ISSN:09182918)

巻号頁・発行日

pp.0959-18, (Released:2018-10-17)

参考文献数

16

被引用文献数

8

---

Anderson-Fabry disease (AFD) is a rare X-linked disorder caused by deficient activity of the lysosomal enzyme α-galactosidase A (α-GAL A). We herein report 10 cases of AFD in 5 families (3 men and 7 women) that were found to have a specific common mutation in R301Q (G-to-A transition in exon 6 [codon 301] resulting in the replacement of a glutamine with an arginine residue). We evaluated their clinical characteristics, residual enzymatic activity, and plasma concentrations of globotriaosylsphingosine (Lyso-Gb3). Although all 10 cases had cardiac and renal manifestations in common, their clinical manifestations were markedly divergent despite the same genetic abnormality.

著者

Yoshie Ojima Kinya Sawada Hiroshi Fujii Tsuyoshi Shirai Ayako Saito Saeko Kagaya Satoshi Aoki Yoichi Takeuchi Tomonori Ishii Tasuku Nagasawa

出版者

The Japanese Society of Internal Medicine

雑誌

Internal Medicine (ISSN:09182918)

巻号頁・発行日

pp.9848-17, (Released:2017-12-27)

参考文献数

47

被引用文献数

7

---

A previously healthy 58-year-old man was admitted for muscle pain and weakness (manual muscle testing [MMT] of 4/4 for upper and lower limbs). We detected elevated levels of inflammatory makers and PR3-anti-neutrophil cytoplasmic antibody (ANCA). Subsequently, the muscle weakness rapidly progressed to an MMT of 2 for all limbs. Magnetic resonance imaging indicated muscle edema, and the CK level increased to 29,998 U/L. mPSL and cyclophosphamide pulse therapy improved the patient symptoms. MMT recovered to 4 for all limbs. A muscle biopsy showed degenerated muscle fibers surrounded by neutrophil-predominant infiltration. In addition, lamina elastic breakdown and fibrinoid necrosis of arterioles were observed. A final diagnosis of microscopic polyangiitis (MPA) limited to the muscles was made.

著者

Yusuke Kobari Tasuku Nagasawa

出版者

一般社団法人 日本内科学会

雑誌

Internal Medicine (ISSN:09182918)

巻号頁・発行日

pp.8704-16, (Released:2017-10-11)

参考文献数

4

被引用文献数

7

---

A previously healthy 77-year-old Japanese man presented with a 2-week history of daily fevers peaking at 38°C, chills, hearing loss, and almost 10 kg of unintentional weight loss over 2 months. Pure tone audiometry showed mixed conductive-sensorineural hearing loss: right, 63.6 dB, left, 80.0 dB. Blood tests after admission showed a high myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) level (>300 U/mL), so we suspected ANCA-related vasculitis. The Japanese Otorhinolaryngology Society has recently been advocating the concept of otitis media with ANCA-associated vasculitis (OMAAV). Our case met the criteria proposed, leading to our diagnosis.