A Unique Presentation of Lupus Podocytopathy with Collapsing FSGS Variant

Document Type

Conference Proceeding

Publication Date

11-1-2023

Publication Title

J Am Soc Nephrol

Abstract

Introduction: Lupus podocytopathy is a rare subset of lupus nephritis seen with nephrotic range proteinuria that presents with findings on kidney biopsy mimicking that of minimal change disease or focal segmental glomerular sclerosis (FSGS). A rare subset of collapsing glomerulopathy can also be seen. This condition has also been linked to the pathologic APOL-1 gene variant. We report a rare case of lupus podocytopathy with collapsing FSGS. Case Description: A 42-year-old African American male with a history of hypertension and systemic lupus erythematosus (on mycophenolate mofetil (MMF) 1.5 g twice daily for non-renal manifestations) presented after outpatient labs showed dramatically increased serum creatinine and BUN. His only reported symptoms were shortness of breath and fatigue. In the ED, blood pressure was 148/84 mmHg. Labs showed serum creatinine of 10.23 mg/dL (baseline of 2.0 mg/dL), BUN of 111, bicarbonate of 9 mmol/L, and hemoglobin of 7.4 g/dL. The urine albumin to creatinine ratio was 1,463.5 mg/g with a urine protein to creatinine ratio of 2.91 g/g. COVID-19, flu A/B, hepatitis panel, EBV, CMV, HIV, blood cultures, and parvovirus were all negative. Kidney biopsy demonstrated acute tubular injury, WHO class II lupus nephritis, and collapsing FSGS in the setting of lupus podocytopathy. APOL-1 gene testing is pending. He was initiated on pulse dose steroids for 3 days, and hemodialysis was initiated due to worsening acidosis. He was continued on his home dose of MMF. He currently remains hemodialysis dependent (declared ESRD). Discussion: Patients with SLE podocytopathy represent less than one percent of all SLE flares, with collapsing FSGS variant even more rare and associated with poor prognosis. In recent years the literature started defining non-HIV-related collapsing glomerulopathy, but no unified data regarding treatment guidelines exist. This patient was treated with a combination of steroids and MMF without success, highlighting a unique presentation of a rarely seen syndrome in lupus nephritis.

Volume

34

First Page

1133

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