Plaquenil-Induced Focal Segmental Glomerulosclerosis
- Glomerular Diseases: Podocyte Biology - I
November 04, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1304 Glomerular Diseases: Podocyte Biology
- Luo, Jack, Alameda Health System, Oakland, California, United States
- Zaman, Warda, East Bay Nephrology Medical Group, Oakland, California, United States
Drugs and toxins that are typically associated with focal segmental glomerulosclerosis (FSGS) include interferons, bisphosphates, anthracyclines, lithium, and calcineurin inhibitors, but hydroxychloroquine is not. We present a case of FSGS in a patient on hydroxychloroquine (HCQ) for treatment of scleroderma.
A 66-year-old female with a history of CREST syndrome with scleroderma was referred to clinic for nephrotic range proteinuria in 2021. She was last seen by rheumatology in the early 2000s. She is originally from Nicaragua, lives in Vallejo, California, and travels back and forth. Since 2020, she has purchased HCQ in Nicaragua which provided relief of joint pain. Physical exam was notable for PIP joint swelling of both hands and bilateral lower extremity edema. Labs showed Cr 0.8-0.9mg/dL, eGFR 70-75ml/min/1.73m2, albumin 3.9g/dL, urinalysis with 2+ protein and no sediment, albumin to creatinine ratio of 1750 mcg/mg in 8/2020. Further work up showed UPCR 4.6, HCV positive but RNA negative, Hep B, RPR, and HIV were negative. SPEP negative, K:L 0.9, dsDNA negative, normal complement, RF negative, Normal CRP. PLA2R antibody was also negative. Kidney biopsy showed myelin figures with widespread foot process effacement in the setting of HCQ use, without any immune complex, paraproteinemic disease, or membranous nephropathy. Patient was diagnosed with secondary FSGS from hydroxychloroquine use. HCQ was stopped, with decrease in proteinuria to 1.2g, almost 4 months since HCQ was held. Irbesartan was continued for medical management of proteinuria.
Zebra bodies, or myelin figures, are intralysosomal inclusion bodies that suggest kidney phospholipidosis, which is a widely accepted feature of hereditary disorders such as Fabry disease, but it has also been seen in patients on drugs such as HCQ. The majority of cases that have demonstrated this have been in patients on HCQ for management of systemic lupus erythematosus. To our knowledge, there are no previous reports of zebra bodies seen on renal biopsies taken from patients on HCQ for treatment of scleroderma or any previous cases of FSGS occurring in the setting of HCQ use. Our case demonstrates the need to consider HCQ as a cause of FSGS in patients evaluated for proteinuria, with negative work up for the usual causes such as viral infections, drugs, or system conditions, but may be on HCQ for another disorder.