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Two cases of Type Ⅲ collagen glomerulopathy and literature review

Fang Yu1, Xuejing Zhu2, Shuguang Yuan3

  • 1Department of Nephrology, Second Xiangya Hospital, Central South University; Institute of Nephrology, Central South University, Changsha 410011. 745623147@qq.com.

Zhong Nan Da Xue Xue Bao. Yi Xue Ban = Journal of Central South University. Medical Sciences
|September 4, 2020
PubMed

Insights

Collagen Type III glomerulopathy presents with varied symptoms like nephrotic syndrome and hypertension. Diagnosis relies on electron microscopy and immunohistochemistry, identifying collagen fiber deposits.

Area of Science:

  • Nephrology
  • Genetics
  • Pathology

Background:

  • Collagen Type III glomerulopathy is a rare kidney disease.
  • Clinical presentations can be atypical, complicating diagnosis.
  • Genetic factors, such as CFHR5 mutations, may play a role.

Purpose of the Study:

  • To analyze clinical, pathological, and genetic features of two collagen Type III glomerulopathy cases.
  • To highlight diagnostic challenges and methods for this rare condition.

Main Methods:

  • Case study analysis of two patients with collagen Type III glomerulopathy.
  • Clinical data review including symptoms, renal function, and treatment outcomes.
  • Pathological examination: light microscopy, immunohistochemistry, and electron microscopy.
  • Genetic analysis for potential disease-causing mutations.

Main Results:

  • Patients presented with nephrotic syndrome, proteinuria, hypertension, and renal dysfunction.
  • Pathology revealed mesangial matrix hyperplasia, sub-endothelial deposits, and positive Type III collagen staining.
  • Electron microscopy showed significant collagen fiber deposition (40-70 nm) in the mesangial matrix and basement membrane.
  • One case showed a potential CFHR5 gene mutation; treatment with an angiotensin II receptor blocker stabilized renal function.

Conclusions:

  • Collagen Type III glomerulopathy exhibits diverse clinical and light microscopy findings.
  • Electron microscopy and immunohistochemistry are crucial for definitive diagnosis.
  • Early diagnosis and targeted treatment may improve patient outcomes, though prognosis varies.

Keywords:
collagen Type Ⅲ glomerulopathyelectron microscopyimmunohistochemistryrenal histopathology

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