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- Published: March 8, 2019
- By: L. Nich Cossey, MD
Collagen type III glomerulopathy is an ultra-rare kidney disease of unknown etiology and pathogenesis with only rare cases reported in the literature, the vast majority being case reports. A few non-controlled, small case series exist that shed light on differences in pediatric and adult presentations and specific geographic patient populations. Type III collagen is typically absent in normal glomeruli, although small amounts have been reported in some advanced glomerular diseases. In patients with collagen type III glomerulopathy, abundant type III collagen is seen within the mesangium and lamina rara interna of the capillary loops and typically occurs in a flocculent...