Fibrillary Glomerulonephritis: A Great Mimicker of Rapidly Progressive Glomerulonephritis.
Fibrillary Glomerulonephritis: A Great Mimicker of Rapidly Progressive Glomerulonephritis.
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DOI：
10.7759/cureus.26001
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发表时间：
2022-06
影响因子：
1.2
通讯作者：
Shafiq, Asad
中科院分区：
文献类型：
--
作者：
Raikar, Manisha;Shafiq, Asad
纤维性肾小球肾炎（FGN）是一种罕见但严重的肾脏疾病，在系膜和/或肾小球毛细血管壁上发现非淀粉样纤维沉积。它最初被认为是特发性的，但最近的研究表明它与自身免疫性疾病、恶性肿瘤和丙型肝炎感染有关。我们报告一例非糖尿病患者表现为长期显微镜下血尿，进行性蛋白尿，高血压和肾功能恶化。肾活检显示上皮下纤维状沉积物大小为17mm，随机定向，电镜显示部分细胞月牙形。直接免疫荧光未见IgG或轻链染色。刚果红染色弱阳性，血清游离kappa/lambda轻链比略高，血清免疫固定未检出单克隆蛋白。我们经验性地使用利妥昔单抗治疗，但没有明显的益处或肾脏没有恢复，最终开始进行血液透析。FGN预后极差，治疗选择很少。我们报道这一病例是为了强调由于这种疾病的罕见性，需要更大的、多中心的研究，通过合作和整合病例报告和病例系列的数据来研究治疗方法。
Fibrillary glomerulonephritis (FGN) is a rare but severe kidney disease found to have non-amyloid fibrillary deposits in the mesangium and/or glomerular capillary wall. It was initially thought to be idiopathic, but recent studies show an association with autoimmune disease, malignancy, and hepatitis C infection. We report a case of a non-diabetic patient presenting with long-standing microscopic hematuria, progressive proteinuria, hypertension, and worsening kidney function. The kidney biopsy demonstrated subepithelial fibrillar deposits of size 17 mm randomly oriented with one partial cellular crescent on electron microscopy. Direct immunofluorescence showed no staining for IgG or light chains. It was weakly positive for Congo red staining with a slightly higher serum free kappa/lambda light chain ratio, but serum immunofixation showed no monoclonal protein detection. We empirically treated with rituximab but with no clear benefit or no renal recovery and eventually started on hemodialysis. FGN has an extremely poor prognosis with very few treatment options available. We report this case to emphasize the need for larger, multi-center studies for treatment approaches with collaborating and consolidating data from case reports and case series due to the rarity of the disease.
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影响因子：
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通讯作者：
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发表时间：
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