Kidney Week

Abstract: FR-PO978

Hydroxychloroquine (HCQ)-Associated Zebra and Myeloid Bodies in Patients with Systemic Lupus Erythematosus (SLE)

Session Information

• Pathology and Lab Medicine - 1
  October 25, 2024 | Location: Exhibit Hall, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Pathology and Lab Medicine

• 1800 Pathology and Lab Medicine

Authors

• Manabe, Shun, Tokyo Women's Medical University, Shinjuku-ku, Tokyo, Japan

Shun Manabe, MD, PhD

• Seki, Momoko, Tokyo Women's Medical University, Shinjuku-ku, Tokyo, Japan

Momoko Seki, DrMed

• Ushio, Yusuke, Tokyo Women's Medical University, Shinjuku-ku, Tokyo, Japan

Yusuke Ushio, MD, PhD

• Kawaguchi, Yuki, Tokyo Women's Medical University, Shinjuku-ku, Tokyo, Japan

Yuki Kawaguchi, MD

• Kobayashi, Shizuka, Tokyo Women's Medical University, Shinjuku-ku, Tokyo, Japan

Shizuka Kobayashi, MD

• Makabe, Shiho, Tokyo Women's Medical University, Shinjuku-ku, Tokyo, Japan

Shiho Makabe

• Ito, Naoko, Tokyo Women's Medical University, Shinjuku-ku, Tokyo, Japan

Naoko Ito, MD

• Kataoka, Hiroshi, Tokyo Women's Medical University, Shinjuku-ku, Tokyo, Japan

Hiroshi Kataoka

• Taneda, Sekiko, Tokyo Women's Medical University, Shinjuku-ku, Tokyo, Japan

Sekiko Taneda, MD, PhD

• Honda, Kazuho, Showa University, Shinagawa-ku, Tokyo, Japan

Kazuho Honda, MD, PhD

• Hoshino, Junichi, Tokyo Women's Medical University, Shinjuku-ku, Tokyo, Japan

Junichi Hoshino, MD, PhD, MPH

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.