Open AccessCollagenofibrotic glomerulopathy - Case report with review of literature
Author(s) -
KC Patro,
Ratan Jha,
Manisha Sahay,
G Swarnalatha
Publication year - 2011
Publication title -
indian journal of nephrology/indian journal of nephrology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.317
H-Index - 24
eISSN - 1998-3662
pISSN - 0971-4065
DOI - 10.4103/0971-4065.78080
Subject(s) - glomerulopathy , medicine , proteinuria , disease , pathology , etiology , pathogenesis , pathological , procollagen peptidase , kidney
Collagenofibrotic glomerulopathy is a rare, idiopathic glomerular disease characterized by abnormal accumulation of type III collagen fibrils within the mesangial matrix and subendothelial space and a marked increase in serum type III procollagen peptide levels. Proteinuria (commonest feature), edema, hypertension, and occasional progression to end-stage renal disease are the various features of this disease. The etiology and pathogenesis remain elusive. There have been reports of the disease running in the family, suggesting the possibility of genetic transmission. We report two cases of this rare entity.