セキ　モモコ   Seki Momoko   関　桃子    所属   医学部 医学科（東京女子医科大学病院）    職種   助教
言語種別	英語
発表タイトル	Hydroxychloroquine (HCQ)-Associated Zebra and Myeloid Bodies in Patients with Systemic Lupus Erythematosus (SLE)
会議名	Kidney Week 2024
主催者	American Society of Nephrology
学会区分	国際学会及び海外の学会
発表形式	ポスター掲示
講演区分	一般
発表者・共同発表者	◎Manabe Shun, Seki Momoko, Ushio Yusuke, Kawaguchi Yuki, Kobayashi Shizuka, Makabe Shiho, Ito Naoko, Kataoka Hiroshi, Taneda Sekiko, Honda Kazuho, Hoshino Junichi,
発表年月日	2024/10/25
国名	アメリカ合衆国
開催地 （都市, 国名）	San Diego, USA
開催期間	2024/10/24～2024/10/27
概要	Background Zebra and myeloid bodies are characteristic findings in Fabry disease. However, the presence of lamellar bodies (LBs) in HCQ-treated patients (HCQ+) occasionally poses a diagnostic challenge in differentiating Fabry disease. Here we aimed to elucidate the histological features of kidney biopsy associated with HCQ treatment. Methods We analyzed SLE patients who underwent kidney biopsy from 2019 to 2024. Electron microscopic (EM) images were evaluated for the maximum number of LBs within involved cells, presence of curvilinear bodies, and intracellular vesicles with osmiophilic granules. We evaluated four Fabry disease cases similarly. Results A total of 37 SLE patients, including 17 HCQ+, underwent kidney biopsy. No case was clinically suggestive for Fabry disease. EM images were taken from 23 patients (Female 20, mean age; 43.5±12.5), including 12 HCQ+. LBs were observed in all 12 HCQ+ (Figure A) and in three (27.2%) cases without HCQ treatment (HCQ-). The maximum number of LBs ranged from one to 49, median (IQR); 2.5 (1-4), in HCQ+, and one, one, and 4 in HCQ-. Of 4 HCQ+ with a single LB, 2 had initiated HCQ less than 10 days prior. A HCQ- with 4 LBs were treated with sertraline; a medicine can be associated with LB. Among Fabry disease patients, the number ranged from 59 to 141 (median, 79.0). Curvilinear bodies were observed in three HCQ+ only. Intracellular vesicles with osmiophilic granules within proximal tubular cells (Figure B) were observed in 7 out of 10 (70%) HCQ+ with images of proximal tubules, and none of (0%) HCQ-. One case underwent globotriaosylceramide 3 staining with a positive result. Three HCQ+, with 4, 5, and 49 LBs, including a case with urinary mulberry cells, underwent genetic testing for α-galactosidase A with normal results. Conclusion The appearance of LBs might be a universal phenomenon associated with HCQ treatment, though the number of LBs was apparently smaller than in Fabry disease. The number of LBs and presence of intracellular vesicles with osmiophilic granules might help differentiate HCQ-associated LBs from Fabry disease.