Collagenofibrotic Glomerulopathy: Clinicopathologic Overview of a Rare Glomerular Disease

Graduate School of Medical and Dental Sciences, Niigata University, Niahi-niigata, Niigata, Japan
American Journal of Kidney Diseases (Impact Factor: 5.9). 05/2007; 49(4):499-506. DOI: 10.1053/j.ajkd.2007.01.020
Source: PubMed

ABSTRACT Collagenofibrotic glomerulopathy is an idiopathic glomerular disease characterized by massive accumulation of atypical type III collagen fibrils within the mesangial matrix and subendothelial space and marked increase in serum type III procollagen peptide levels. The disease is extremely rare, with most cases reported in Japan. The cause and pathogenesis are entirely elusive. Some cases were described in families; hence, a genetic mode of transmission, mostly by an autosomal recessive trait, has been assumed. Controversy exists about whether the glomerulopathy is a primary renal disease or manifestation of systemic disease. Proteinuria is a cardinal manifestation of this disease. Clinically, patients present with edema and hypertension and often progress to end-stage renal disease. A definite diagnosis can be established when typical histological findings are supported by immunohistochemistry for specific collagen types and electron microscopy with special staining methods. No specific treatment is available.

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    • "Elevated serum levels of PIIINP in Col3GP affected dogs compared to healthy age-matched controls were observed in the present study. Increased serum PIIINP is well described in Col3GP affected humans [18-22], but has so far not been reported in animals. Regarding the control group, the younger puppies had higher serum PIIINP concentrations than the older ones, and the serum levels declined with increasing age at sampling. "
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    ABSTRACT: Collagen type III glomerulopathy (Col3GP), also known as collagenofibrotic glomerulonephropathy, is a rare renal disease with unknown pathogenesis that occurs in animals and humans. We recently described a naturally occurring canine autosomal recessive model of Col3GP, and the aim of the present work was to study the clinical features of canine Col3GP and compare with the human phenotype. In humans two different clinical syndromes with different age at onset (child- or adulthood) have been observed. In children a more aggressive course with familial occurrence is described, characterized by progressively increasing proteinuria, nephrotic syndrome, hypertension and chronic renal failure. A markedly increased serum level of the aminoterminal propeptide of type III procollagen (PIIINP) is considered a useful marker for the disease. Since Col3GP and concurrent hypocomplementemia have been observed in humans, we also aimed to investigate if hypocomplementemia was present in Col3GP affected dogs. A litter consisting of seven puppies, four Col3GP affected and three healthy unaffected, was observed from the day of birth until the affected puppies developed a mild or moderate renal azotemia. During the period of observation growth retardation, increasing blood pressure, progressive proteinuria, azotemia, hypoalbuminemia, hypercholesterolemia and increased serum PIIINP were observed in all the affected dogs. Hypocomplementemia was not detected. Affected dogs were euthanized between 109 and 144 days of age, and pathological examinations revealed ascites and massive glomerular accumulations of collagen type III, consistent with Col3GP. Dogs with Col3GP develop juvenile chronic renal failure, preceded by nephrotic syndrome, elevated serum PIIINP and hypertension, thus have similar clinical features as the juvenile Col3GP in humans. Further studies of this naturally occurring canine phenotype may provide more information on the pathogenesis and genetics of Col3GP in both animals and humans.
    BMC Veterinary Research 10/2013; 9(1):218. DOI:10.1186/1746-6148-9-218 · 1.78 Impact Factor
    • "It is usually sporadic and when noticed in siblings the entity has been considered to be an autosomal recessive trait. There has been a speculation whether it is a primary renal disease or manifestation of a systemic disease, i.e., the abnormal collagen is from within the glomerulus or from an extrarenal source.[3] "
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    ABSTRACT: 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.
    Indian Journal of Nephrology 04/2011; 21(1):52-5. DOI:10.4103/0971-4065.78080
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    • "Based on the archive of renal biopsies at Nephopathology Service at General Pathology at the Federal University of Triângulo Mineiro (UFTM), we have identified three cases of CG that occurred from 2000 to 2007. There hadn't been any cases reported in South America until that time, since the great majority of cases had occurred in Japan [5]. "
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    ABSTRACT: We are reporting the first collagenofibrotic glomerulopathy (CG) in South America. So, this collagen type III glomerulopathy is not limited to Japan but may be found throughout the world. We describe three patients that presented some factors in common, such as sex, age and the presence of non-nephrotic proteinuria associated with microscopic hematuria. The findings with the immunofluorescence microscopy, of immunoglobulins, and components of the complement were usually negative. The picrosyrius staining showed the presence of reddish material in the mesangium, when it was seen under standard microscopy; however, when it was seen with birefringence, it became greenish under polarized light, showed the collagen found in this area of the glomerulus. The identification of CG was made through electronic microscopic scanning, and curved and disorganized fibers were found. These cases are the first from South America to be reported, and they are about an idiopathic renal disease that is not related to any specific races or locations. The reports contribute to a better understanding of this disease, which although not so prevalent, should be considered as an importantly differential diagnostic of cases of proteinuria.
    Diagnostic Pathology 09/2009; 4(1):33. DOI:10.1186/1746-1596-4-33 · 2.60 Impact Factor
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