Jagadish Ashwin, Vedantam Venkata, Vedantam Neethu, Magacha Hezborn M
Internal Medicine, East Tennessee State University Quillen College of Medicine, Johnson City, USA.
Infectious Diseases, East Tennessee State University Quillen College of Medicine, Johnson City, USA.
Cureus. 2023 Feb 16;15(2):e35068. doi: 10.7759/cureus.35068. eCollection 2023 Feb.
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.
纤维性肾小球肾炎是一种罕见疾病，其特征为肾小球内出现非分支状纤维积聚，导致血尿、蛋白尿和高血压。它常与恶性肿瘤相关，但病因不明。一名66岁有外阴鳞状细胞癌病史的白人女性，在新发进行性肾功能不全和肾病范围蛋白尿的情况下，出现了一个月的无痛性血尿。全面评估，包括肾活检及对DnaJ热休克蛋白家族成员B9（DNAJB9）进行染色，明确诊断为纤维性肾小球肾炎。该患者最初接受了利妥昔单抗和泼尼松治疗。不幸的是，在接下来的一个月里她的肾功能持续下降，需要重新住院并开始血液透析。据我们所知，这是首例描述外阴鳞状细胞癌与纤维性肾小球肾炎之间关联的病例。
