Fibrillary Glomerulonephritis in a Patient With Vulvar Squamous Cell Carcinoma.

ABSTRACT

Fibrillary glomerulonephritis is a rare condition characterized by glomerular accumulation of non-branching fibrils, leading to hematuria, proteinuria, and hypertension. It is often associated with malignancy but has no known cause. A 66-year-old Caucasian female with a history of vulvar squamous cell carcinoma presented with one month of painless hematuria in the setting of new-onset progressive renal dysfunction and nephrotic range proteinuria. Comprehensive evaluation, including renal biopsy with staining for DnaJ heat shock protein family member B9 (DNAJB9), provided a definitive diagnosis of fibrillary glomerulonephritis. The patient initially received rituximab and prednisone therapy. Unfortunately, her renal function continued to decline over the next month, requiring re-hospitalization with initiation of hemodialysis. To our knowledge, this is the first case describing an association between vulvar squamous cell carcinoma and fibrillary glomerulonephritis.