Abstract: FR-PO445
Renal Vein Thrombosis Leading to the Diagnosis of Nephrotic Syndrome
Session Information
- Pediatric Nephrology - I
November 04, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Genetic Diseases of the Kidneys
- 1102 Genetic Diseases of the Kidneys: Non-Cystic
Authors
- Leonard, Kathryn, Nassau University Medical Center, East Meadow, New York, United States
- Alam, Ishmam Y., Nassau University Medical Center, East Meadow, New York, United States
- Cadet, Bair, Nassau University Medical Center, East Meadow, New York, United States
- Singh, Jaswinder P., Nassau University Medical Center, East Meadow, New York, United States
Introduction
Primary MN is the most common cause of idiopathic nephrotic syndrome. We present the case of a 31-year-old Hispanic male with no prior past medical or family history, presenting with renal vein thrombosis and lab dysfunction, concerning for nephrotic syndrome. Further workup of kidney biopsy and serum antibody levels revealed the cause to be anti-PLA2R-mediated.
Case Description
31-year-old Hispanic male with no past medical or family history presented with chest pain, dyspnea and swelling in his face and legs for one day. He reported recent pulmonary embolism and was discharged on anticoagulation therapy. He reported the chest pain and dyspnea felt similar to his prior hospitalization but the swelling was new. Physical exam revealed pitting edema to bilateral lower extremities and periorbital edema.
Pan CT revealed subsegmental pulmonary emboli, renal vein thrombus and ascites. Laboratory workup revealed hypoalbuminemia less than 1.3 gm with total protein 3.4 g/dL. Lipid panel revealed elevated total cholesterol of 424 mg/dL and LDL of 339 mg/dL. Initial urinalysis showed proteinuria of >300 mg/dL, with spot urine protein creatinine ratio of 6.5, confirmed by 24-hour urine protein and creatinine ratio of 23.5.
Serum PLA2R antibody panel was positive with a level of 47 RU/mL. Thrombophilic workup was unremarkable. Kidney biopsy with immunofluorescence staining for IgG, C3, kappa and lambda chains and PLA2R were positive, confirming primary MN
Discussion
While renal vein thrombosis is common, thromboembolic events preceding classic symptoms is unusual. MN is associated with lower extremity edema, periorbital edema, proteinuria and ascites. Uniquely, our patient presented with shortness of breath and chest pain. This case emphasizes the importance of imaging and highlights the role of anti-PLA2R antibody levels as a clinical predictor.
Antibodies to PLA2R have been reported in 70% of patients with primary MN. Lower titer is associated with better remission rates. One study among patients with biopsy-proven MN revealed that high PLA2R antibody levels were linked with active disease and higher likelihood of declining renal function. These levels can dictate immunosuppressive therapies among patients. Additionally changes in circulating PLA2R antibodies occur more rapidly than changes in proteinuria, making it useful to monitor disease activity and treatment response.