Bao Hao, Chen Huiping, Zhu Xiaodong, Xu Feng, Zhu Maoyan, Zhang Minchao, He Qian, Zeng Caihong, Liu Zhihong
National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.
Histopathology. 2015 Oct;67(4):568-76. doi: 10.1111/his.12685. Epub 2015 Apr 21.
We report nine Chinese patients with collagen type III glomerulopathy.
Two males and seven females were studied, ranging in age from 21 to 67 years. Proteinuria and hypertension were the most common symptoms, with incidences of 88.9 and 77.8%, respectively. Two patients had abnormal renal function. Their histological appearances varied. Massive eosinophilic and weakly periodic acid-Schiff (PAS)-positive substances were deposited along the capillary loops and in the mesangial area in three cases, while others had thickened capillary walls with a chain-like structure or double-contour appearance of the PAS- and silver-stained sections. Immunofluorescence analysis showed the abundant deposition of collagen type III. Electron microscopy revealed massive scattered or bundle-shaped fibre-like materials in the subendothelial and mesangial areas. During follow-up, 44.4% of the patients suffered a doubling of serum creatinine. The level of serum creatinine at biopsy was an independent predictor of this doubled serum creatinine value.
Collagen type III deposits in the subendothelial and mesangial areas. Some patients show global nodular lesions, while others show subtle changes only via PAS/silver staining. Proteinuria and hypertension are the most common symptoms, and the serum creatinine level at biopsy is an independent predictor of the doubling of serum creatinine during follow-up.
