Patel Prem S, Singh Prit P, Krishna Amresh, Kumar Om
Department of Nephrology, Indira Gandhi Institute of Medical Science, Patna, Bihar, India.
Department of Microbiology, All India Institute of Medical Sciences, Patna, Bihar, India.
Indian J Nephrol. 2025 Mar-Apr;35(2):298-301. doi: 10.25259/ijn_392_23. Epub 2024 Jun 4.
Zebra bodies are intralysosomal lamellar inclusion bodies. It is accepted as a specific feature of Fabry disease. However, it has been reported in many hereditary and acquired conditions. We are reporting Zebra bodies in the kidneys of cases with Rheumatoid Arthritis and hydroxychloroquine-induced phospholipidosis. Case 1: A 55-year-old male presented with hypertension and renal dysfunction. Serum ANA and anti-CCP antibodies were positive. A kidney biopsy revealed chronic tubulointerstitial nephritis with Zebra Bodies in the podocytes. Genetic analysis was negative for Fabry disease. Case 2: A 34-year-old female with Systemic Lupus Erythematosus on Hydroxychloroquine for a year presented with subnephrotic proteinuria. Serum ANA and anti-dsDNA antibodies were positive. Electron microscopy showed lamellated osmiophilic inclusion bodies in the tubular and visceral epithelial cells. Thus, Zebra bodies are not pathognomonic for Fabry disease and Rheumatoid Arthritis should also be considered in the differential diagnosis, particularly if family or drug history is negative.
斑马小体是溶酶体内层状包涵体。它被认为是法布里病的一个特异性特征。然而，在许多遗传性和获得性疾病中都有其报道。我们报告了类风湿关节炎和羟氯喹诱导的磷脂沉积症患者肾脏中的斑马小体。病例1：一名55岁男性，表现为高血压和肾功能不全。血清抗核抗体和抗环瓜氨酸肽抗体呈阳性。肾脏活检显示慢性肾小管间质性肾炎，足细胞中有斑马小体。法布里病的基因分析为阴性。病例2：一名34岁女性，系统性红斑狼疮患者，服用羟氯喹一年，出现亚肾病性蛋白尿。血清抗核抗体和抗双链DNA抗体呈阳性。电子显微镜显示肾小管和内脏上皮细胞中有层状嗜锇性包涵体。因此，斑马小体并非法布里病所特有，在鉴别诊断中也应考虑类风湿关节炎，尤其是在家族史或用药史为阴性的情况下。
