Abstract: TH-PO195

Spontaneous Cerebral Venous Thrombosis as a Presenting Manifestation of Secondary Membranous Nephropathy

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Tandukar, Srijan, University of Pittsburgh Medical Center , Pittsburgh, Pennsylvania, United States
  • Rondon Berrios, Helbert, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States
Background

Membranous nephropathy is one of the most common causes of nephrotic syndrome in adults and accounts for the glomerulopathy associated with the highest rate of thromboembolic complications. We present a case of an otherwise healthy man who presented with spontaneous cerebral venous thrombosis and ultimately was diagnosed with a form of secondary membranous nephropathy.

Methods

A 40 year-old previously healthy man presented with progressively worsening generalized headache and nausea. He had been taking occasional ibuprofen for headaches for 2 weeks. He was afebrile and hemodynamically stable at presentation. On examination, he was in moderate distress due to headaches without neck stiffness, temporal artery tenderness or other focal neurological deficit. Laboratory data revealed normal blood counts, chemistry profile and renal function but low serum albumin of 2.3 g/dL, high total cholesterol of 263 mg/dL and high LDL of 191 mg/dL. Urine protein-to-creatinine ratio was elevated at 10 g/g. CT angiography of the brain revealed bilateral transverse venous sinus thrombosis. Patient was initiated on warfarin with heparin bridging. Thrombophilia workup could not be performed since acute thrombosis and warfarin reduce levels of antithrombin, protein C, and protein S. However, antiphospholipid antibodies were negative. Serological workup demonstrated low normal serum complement (C3 95, C4 22) with positive ANA (1:320), anti-U1RNP, anti-SSA, anti-Smith and anti-dsDNA. Hepatitis screen was negative. Kidney biopsy was performed. Light microscopy revealed a diffuse thickening of the glomerular basement membrane with “spikes”. Immunofluorescence studies showed a negative PLA2R staining and a classic “full house” staining pattern (IgG1-4, C3, C1q). Electron microscopy revealed subepithelial, subendothelial and mesangial deposits with tubuloreticular inclusions. Based on the above findings a diagnosis of mixed connective tissue disease with lupus overlap was made and patient was initiated on prednisone and mycophenolate mofetil.

Conclusion

Cerebral venous thrombosis is a rare but potentially fatal complication of nephrotic syndrome. Increased risk of thromboembolism has been classically demonstrated in primary, but is also commonly seen in secondary membranous nephropathy. The risk increases with the level of decline in serum albumin.