Zebra bodies without Fabry disease or hydroxychloroquine
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IMAGES IN NEPHROLOGY
Zebra bodies without Fabry disease or hydroxychloroquine Keita Kadosawa1 · Takashi Morikawa1 · Yoshio Konishi1 Received: 9 June 2020 / Accepted: 26 August 2020 © Japanese Society of Nephrology 2020
Keywords Zebra body · Anti-phospholipid antibody · Lupus nephritis · Fabry disease · Hydroxychloroquine A 29-year-old man was diagnosed with systemic lupus erythematosus (SLE) because of polyarthritis, pancytopenia, proteinuria with microhematuria, and positivity for antinuclear antibodies and anti-phospholipid antibody. A renal biopsy before treatment showed lupus nephritis class II + V (Fig. 1a, b, c). Glomerulosclerosis and interstitial fibrosis were scarce, and vacuolated podocytes were inconspicuous by light microscopy. However, electron microscopy revealed laminated zebra bodies in one podocyte (Fig. 1d, e), which is typical of Fabry disease [1]. The patient had no personal or family history supporting a diagnosis of Fabry disease.
A-galactosidase activity was normal at 15 nmol/h/mL. Some reports show that patients with lupus nephritis develop zebra bodies after receiving hydroxychloroquine [2, 3], but our patient has not taken this drug. Although the relationship between phospholipidosis and anti-phospholipid antibodies or SLE was suggested in some case reports [4, 5], the detailed mechanism remains unknown. This is a rare case of anti-phospholipid antibody positive lupus nephritis with zebra bodies without Fabry disease or hydroxychloroquine. The cause of zebra bodies is unclear, and further investigation is needed to clarify it.
* Takashi Morikawa taka‑[email protected]‑hp.or.jp 1
Department of Nephrology and Hypertension, Osaka City General Hospital, 2‑13‑22, Miyakojima‑hondori, Miyakojima‑ku, Osaka 534‑0021, Japan
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Clinical and Experimental Nephrology
Fig. 1 a Immunofluorescence microscopy reveals that mesangial and granular subepithelial immune deposits are stained for IgG. C3, IgA, IgM and C1q are also positive in the same lesion. b Electron-dense deposits (white arrows) in the mesangial area are observed. c Small electron-dense deposits (white arrows) in the basement membrane are
seen with thickening of capillary walls. These findings are consistent with International Society of Nephrology/Renal Pathology Society Classification II + V. d Laminated “zebra bodies” are apparent in a podocyte. e Higher magnification of the zebra bodies in a podocyte are observed
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References
Conflict of interest The authors have declared that no conflict of interest exists.
1. Gubler MC, Lenoir G, Grunfeld JP, Ulmann A, Droz D, Habib R. Early renal changes in hemizygous and heterozygous patients with Fabry’s disease. Kidney Int. 1978;13(3):223–35. 2. Bojic M, Kozakowski N, Becede M, Kerschbaumer A, Bobacz K. The Case I Myeloid bodies in the kidney biopsy of a patient with systemic lupus erythematosus. Kidney Int. 2017;92(1):271–2.
Ethical approval This article does not contain any studies with animals performed by any of t
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