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The Copycat Disease: A Case of Lupus Podocytopathy
The Copycat Disease: A Case of Lupus Podocytopathy
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This case describes a 44-year-old woman with a history of trigeminal neuralgia who first presented to the emergency department with 10 days of bilateral lower-extremity edema and abdominal distension. She had no chest pain, shortness of breath, fever, cough, dysuria, or orthopnea. Exam showed mild bilateral ankle pitting edema with otherwise normal cardiopulmonary and abdominal findings. Initial workup revealed marked nephrotic-range proteinuria, hypoalbuminemia (albumin 1.5), elevated BNP, hematuria, and bilateral pleural effusions on CT, leading to a diagnosis of nephrotic syndrome and outpatient nephrology follow-up.<br /><br />Two weeks later, she returned with spasms in her hands and legs and was admitted for worsening acute kidney injury and evaluation for systemic lupus erythematosus (SLE), given a strong family history of lupus on her maternal side. Inpatient renal biopsy showed findings consistent with minimal change disease, including diffuse podocyte foot process effacement on electron microscopy and no immune complex deposition typical of lupus nephritis. Immunofluorescence demonstrated tissue ANA correlating with a positive serum ANA. These findings were interpreted as lupus podocytopathy (LP), a rare lupus-related nephrotic syndrome that can mimic minimal change disease or focal segmental glomerulosclerosis.<br /><br />She was treated with prednisone, bumetanide, and warfarin for thrombosis prophylaxis, then later started on hydroxychloroquine as an outpatient. Her urine protein-to-creatinine ratio improved on follow-up. The case highlights that lupus podocytopathy should be considered in patients with SLE or suspected SLE who present with nephrotic syndrome, since it is uncommon, often steroid-responsive, and may require anticoagulation because of increased thrombotic risk.
Asset Subtitle
Rina Joshi
Meta Tag
Author List
John H. Oneill Jr., Maryah Mansoor, Rina Joshi
Category
Clinical Vignettes
Concept
Nephrotic Syndrome
Concept
Lupus Podocytopathy
Concept
Proteinuria
Concept
Systemic Lupus Erythematosus
Concept
Kidney Biopsy
Distinguished
Non-Finalist
Presenter Organization
Christiana Care
Presenting Author
Rina Joshi
Track
Adult
Keywords
lupus podocytopathy
nephrotic syndrome
minimal change disease
systemic lupus erythematosus
proteinuria
hypoalbuminemia
acute kidney injury
renal biopsy
podocyte foot process effacement
warfarin anticoagulation
Nephrotic Syndrome
Lupus Podocytopathy
Proteinuria
Systemic Lupus Erythematosus
Kidney Biopsy
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