Department of Nephrology, Hospital Central Defense Gomez Ulla, 28047 Madrid, Spain.
Department of Nephrology, Guadalajara Center Dialysis, AVERICUM, 19003 Guadalajara, Spain.
Med Sci (Basel). 2024 May 15;12(2):25. doi: 10.3390/medsci12020025.
Phospholipidosis is a rare disorder which consists of an excessive intracellular accumulation of phospholipids and the appearance of zebra bodies or lamellar bodies when looking at them using electron microscopy. This disease is associated with certain genetic diseases or is secondary to drugs or toxins. Drug-induced phospholipidosis encompasses many types of pharmaceuticals, most notably chloroquine, amiodarone or ciprofloxacin. Clinically and histologically, renal involvement can be highly variable, with the diagnosis not being made until the zebra bodies are seen under an electron microscope. These findings may require genetic testing to discount Fabry disease, as its histological findings are indistinguishable. Most of the chemicals responsible are cationic amphiphilic drugs, and several mechanisms have been hypothesized for the formation of zebra bodies and their pathogenic significance. However, the relationship between drug toxicity and phospholipid accumulation, zebra bodies and organ dysfunction remains enigmatic, as do the renal consequences of drug withdrawal. We present, to our knowledge, the first case report of acute renal injury with a monoclonal gammopathy of renal significance, lesions, and sclerodermiform syndrome, with zebra bodies that were associated with the initiation of a hydroxychloroquine and amiodarone treatment, as an example of drug-induced-phospholipidosis.
磷脂病是一种罕见的疾病，其特征是细胞内磷脂过度积累，并且在使用电子显微镜观察时会出现斑马体或板层体。这种疾病与某些遗传疾病有关，或者是药物或毒素的继发症。药物诱导的磷脂病包括许多类型的药物，最著名的是氯喹、胺碘酮或环丙沙星。在临床上和组织学上，肾脏受累的情况可能变化很大，只有在电子显微镜下观察到斑马体时才能做出诊断。这些发现可能需要进行基因检测以排除法布里病，因为其组织学表现无法区分。大多数负责的化学物质是阳离子两亲性药物，并且已经提出了几种形成斑马体及其致病意义的机制。然而，药物毒性与磷脂积累、斑马体和器官功能障碍之间的关系仍然是个谜，药物停药的肾脏后果也是如此。我们提出了一个病例报告，这是我们所知的首例与羟氯喹和胺碘酮治疗开始相关的急性肾损伤、单克隆丙种球蛋白病、肾意义、病变和硬皮病样综合征、斑马体的病例，这是药物诱导的磷脂病的一个例子。
