Matthai Smita Mary, Mohapatra Anjali, Duhli Neelaveni, David Vinoi G, Varughese Santosh
Department of GI Sciences, Central Electron Microscopy Facility, Wellcome Trust Research Laboratory, Vellore, Tamil Nadu, India.
Department of Nephrology, Christian Medical College, Vellore, Tamil Nadu, India.
Indian J Pathol Microbiol. 2020 Feb;63(Supplement):S47-S49. doi: 10.4103/IJPM.IJPM_341_18.
Collagenofibrotic glomerulopathy (CFG) is a rare idiopathic kidney disease characterized by abnormal deposition of atypical Type III collagen fibers in the glomerulus causing subendothelial and mesangial expansion, manifesting as progressive renal dysfunction accompanied by proteinuria. The majority of CFG cases reported in literature are from Japan where this disease entity was initially recognized. There is an increased awareness and diagnosis of this rare renal disease in India with the recent increase in utilization of electron microscopy (EM) in clinical diagnostic settings. We describe a 28-year-old Bangladeshi woman who presented with hypertension and nephrotic range proteinuria not amenable to treatment with steroids and cyclophosphamide, whose renal biopsy demonstrated diagnostic ultrastructural features of CFG. This illustrative case is presented to highlight the role of EM analysis for diagnostic accuracy in renal biopsy evaluation in addition to demonstrating the unusual renal biopsy findings of this rare entity.
胶原纤维性肾小球病（CFG）是一种罕见的特发性肾脏疾病，其特征是肾小球中非典型III型胶原纤维异常沉积，导致内皮下和系膜扩张，表现为进行性肾功能不全并伴有蛋白尿。文献报道的大多数CFG病例来自日本，该病最初在日本被认识。随着电子显微镜（EM）在临床诊断中的应用增加，印度对这种罕见肾脏疾病的认识和诊断也有所增加。我们描述了一名28岁的孟加拉国女性，她患有高血压和肾病范围蛋白尿，对类固醇和环磷酰胺治疗无效，其肾活检显示了CFG的诊断性超微结构特征。除了展示这种罕见疾病不寻常的肾活检结果外，本病例还强调了EM分析在肾活检评估中对诊断准确性的作用。
