Collagen Type III Glomerulopathy

• Type of Material: Article
• Authors: AnnaLisa V. Wilson, Johns Hopkins School of Medicine; Francesca Costigliolo, Johns Hopkins School of Medicine; Alton Farris III, Emory University; Rohan Rengen, Frederick Memorial Hospital; Lois J. Arend, Johns Hopkins School of Medicine
• Language: English
• Date: 2021-01-01
• Publisher: Elsevier
• Publication Version: Final Published Version
• Copyright Statement: © 2021 International Society of Nephrology. Published by Elsevier Inc.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1016/j.ekir.2021.03.887
• Title of Journal or Parent Work: Kidney International Reports
• Volume: 6
• Issue: 6
• Start Page: 1738
• End Page: 1742
• Keywords: glomeruli; accumulation; Collagen type III glomerulopathy (CG); extracellular matrix; collagenofibrotic glomerulopathy; nail–patella syndrome (NPS)
• Research Categories: Biology, Cell

Abstract

Collagen type III glomerulopathy (CG), also known as collagenofibrotic glomerulopathy, is caused by accumulation of collagen type III in glomeruli. Collagen type III is not a normal constituent of the extracellular matrix of glomeruli, but it is found in the interstitium and vessel walls. Clinical features of CG include proteinuria (often in the nephrotic range), edema, and hypertension.1,2 The condition can progress to end-stage kidney disease, requiring dialysis or renal transplantation. While initial reports of CG were largely in patients of Asian descent, subsequent cases in patients of European descent were published.1,3, 4, 5, 6, 7 Approximately 100 cases have been reported in the literature (Supplementary Table S1). We report the first case of CG in an African American patient, suggesting that the condition may be an unrecognized cause of proteinuria in patients of African American descent. Furthermore, our case, as well as 2 of the earliest reported cases,3,8 documents CG in a female who presented with a history of proteinuria associated with pregnancy, highlighting the need to include CG in the differential of female patients who develop new-onset or worsening proteinuria and hypertension with pregnancy. This rare disease has overlapping clinical features with many conditions that can produce nephrotic-range proteinuria, leading to a clinical diagnostic dilemma (Table 1). Likewise, the histologic pattern can be confused with conditions that cause a nodular or membranoproliferative glomerular change, such as amyloidosis and/or fibrillary glomerulonephritis, diabetic nephropathy, fibronectin glomerulopathy, light chain deposition disease, and thrombotic microangiopathy, as well as other conditions with collagen deposition, such as hereditary multiple exostoses and nail–patella syndrome (NPS).