Hydroxychloroquine-Induced Renal Phospholipidosis

Renal phospholipidosis is a rare condition in which excessive phospholipids accumulate in the lysosomes of the renal cells, leading to renal dysfunction. Historically, phospholipidosis has been described in the context of inherited disorders of lysosomal phospholipid metabolism such as Fabry disease. It is now known that many medications can cause excessive phospholipid accumulation, leading to cellular dysfunction. We present a case of hydroxychloroquine-induced renal phospholipidosis in a 79-year-old female patient with Sjogren's disease who had been on hydroxychloroquine for several years and developed nephrotic range proteinuria. She underwent a renal biopsy that showed myeloid (lamellar) bodies within podocytes, a morphological sign of phospholipidosis. Immunofluorescence staining for other potential causes was negative, making it a case of drug-induced phospholipidosis. This patient also had underlying chronic kidney disease stage II. This case raises a question of whether an underlying impaired renal function contributes to the risk of developing drug-induced phospholipidosis, as well as the importance of timely and accurate diagnosis to prevent further harm and to increase the chances of renal recovery.