The Case | Myeloid bodies in the kidney biopsy of a patient with systemic lupus erythematosus

Bojić, Marija; Kozakowski, Nicolas; Bécède, Manuel; Kerschbaumer, Andreas; Bobacz, Klaus

doi:10.1016/j.kint.2016.12.025

Cited by 7 publications

(6 citation statements)

References 3 publications

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“…The existence of curvilinear bodies has been reported to be a distinguishing factor. 5 , 8 , 9 , 12 However, not all previously reported cases 10 , 11 , 13 , 14 , 15 and none of the present cases exhibited curvilinear bodies. Another histologic finding was the presence of intracellular vesicles with osmiophilic granules in the cytoplasm of some podocytes ( Fig S1A ) and all proximal tubular epithelial cells ( Fig S1B ).…”

Section: Discussioncontrasting

confidence: 58%

“…Absence or reduced α-galactosidase activity results in the accumulation of glycosphingolipids, a subtype of glycolipids, which ultrastructurally form zebra bodies. 6 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 Phospholipases A and C metabolize phospholipids, and their reduced activity results in an accumulation of osmiophilic phospholipids. 8 , 10 , 13 , 16 Therefore, inhibition of these enzymes by chloroquine and hydroxychloroquine is presumed to result in the formation of zebra bodies and osmiophilic granules.…”

Section: Discussionmentioning

confidence: 99%

“…In previous studies investigating zebra bodies during chloroquine or hydroxychloroquine treatment by kidney biopsy, enzyme activity or genetic analyses were performed in 7 of the 8 patients 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 ( Table S1 ). All tested patients did not have Fabry disease.…”

Section: Discussionmentioning

confidence: 99%

“…All tested patients did not have Fabry disease. 8 , 9 , 10 , 11 , 12 , 14 , 15 In 1 patient who did not undergo enzyme activity or genetic analyses, a repeat kidney biopsy was performed after the cession of hydroxychloroquine treatment, which indicated the disappearance of zebra bodies. 13 In our cases, the patients lacked family histories and symptoms suggestive of Fabry disease, including cornea verticillata, which is reported to be observed in most women with Fabry disease.…”

Section: Discussionmentioning

confidence: 99%

“…Chloroquine and hydroxychloroquine protect against kidney damage in lupus nephritis. 1 , 2 , 7 Although rare, zebra body accumulation in the kidney has been reported in patients treated with chloroquine and hydroxychloroquine 6 ( Table S1 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 ). Detection of such unusual intracellular structures requires differential diagnosis from Fabry disease, a condition that is typically characterized by zebra bodies.…”

Section: Introductionmentioning

confidence: 99%

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Lupus Nephritis and Hydroxychloroquine-Associated Zebra Bodies: Not Just in Fabry Disease

et al. 2021

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Zebra bodies in kidney biopsy specimens are widely accepted as a specific feature of Fabry disease but they can also be present in a drug-induced mimic of Fabry disease, phospholipidosis. Chloroquine and hydroxychloroquine may both induce zebra body formation and kidney phospholipidosis. However, the frequency and clinical significance of such changes remain unknown. We report 5 serial kidney biopsy cases diagnosed as lupus nephritis during hydroxychloroquine administration. All 5 patients exhibited a few, but varying amounts, of zebra bodies in glomerular intrinsic cells, that is, podocytes, parietal epithelial cells, mesangial cells, and endothelial cells. Most of the zebra bodies detected were subtle, though certainly recognizable; these zebra bodies were much smaller than those observed in Fabry disease. Zebra bodies were not observed in patients with lupus nephritis in the absence of chloroquine or hydroxychloroquine administration. All patients with lupus nephritis who received hydroxychloroquine achieved complete remission during continuous use of hydroxychloroquine, though kidney toxicity of drug-induced phospholipidosis might be masked by immunosuppression. Based on this small series of cases, we speculate that the hydroxychloroquine-associated manifestation of zebra bodies and phospholipidosis in the kidney may be frequent phenomena and may have only a subclinical influence on kidney function, at least in the short term.

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