Collagenofibrotic Glomerulopathy

Collagenofibrotic glomerulopathy or LMX1B-associated nephropathy is a rare disease in which type III collagen accumulates in the glomeruli. We herein report a 64-year-old Japanese woman with an elevated serum creatinine level and persistent proteinuria for 7 years. An electron microscopic study usin...

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Detalles Bibliográficos
Autores principales: Miyake, Masato, Katayama, Kan, Ehara, Takashi, Sado, Yoshikazu, Nawa, Shunpei, Murata, Tomohiro, Mizutani, Yasuhide, Joh, Kensuke, Ito, Masaaki, Dohi, Kaoru
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2020
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8024945/
https://www.ncbi.nlm.nih.gov/pubmed/33055489
http://dx.doi.org/10.2169/internalmedicine.6090-20
Descripción
Sumario:Collagenofibrotic glomerulopathy or LMX1B-associated nephropathy is a rare disease in which type III collagen accumulates in the glomeruli. We herein report a 64-year-old Japanese woman with an elevated serum creatinine level and persistent proteinuria for 7 years. An electron microscopic study using tannic acid showed curved and frayed collagen fibers within mesangial and subendothelial regions compatible with type III collagen depositions. The distribution of type IV collagen α1-6 chains was normal. Since no pathogenic mutations were identified in the LMX1B gene, she was diagnosed with collagenofibrotic glomerulopathy and treated with angiotensin II receptor blocker and calcium antagonist to control her blood pressure.

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