Zebra bodies without Fabry disease or hydroxychloroquine

Excerpt

A 29-year-old man was diagnosed with systemic lupus erythematosus (SLE) because of polyarthritis, pancytopenia, proteinuria with microhematuria, and positivity for anti-nuclear 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.

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