Collagen Type III Glomerulopathy: A New Idiopathic Glomerular Disease

Abstract

A new type of idiopathic glomerular disease is reported in a 49-year-old Italian woman who presented with uncharacteristic renal symptoms, i.e., hypertension and slight proteinuria. Clinical investigation excluded a familial renal disease and more specifically nail-patella syndrome. Diagnostic renal biopsy by light microscopy showed a picture similar to membranoproliferative glomerulonephritis. The enlarged glomeruli were lobulated, the peripheral basement membranes were thickened by the deposition of light-microscopically undefined material, cell proliferation was lacking. By electron microscopy, the material was nonhomogenous, partly granular partly fibrillar, containing typical collagen fibers. The latter were identified as collagen type III, to a lesser extent collagen type I. Review of the literature resulted in 12 similar or identical cases reported from Japan and one additional case reported in a white American female. Evidence of systemic disease is lacking. Etiology and pathogenesis are elusive. A progressive deterioration of renal function must be expected. Collagen type III glomerulopathy is suggested as term of this new type of idiopathic glomerular disease.

Modulation of human mesangial cell behaviour by extracellular matrix components—the possible role of interstitial type III collagen

“…It is reasonable, therefore, to conclude that GAGs and proteoglyeans, such as heparin and heparan sulphate, and type III collagen produced under pathological conditions may exert a negative feedback effect on MC proliferation. In patients with collagen type III nephropathy [19] or primary glomerular fibrosis [20], primary diffuse. tobular accumulation of type III collagen fibres in the glomerulus without MC proliferation has been reported to be a eharacteristic feature.…”

Pathological Characterization of Collagenofibrotic Glomerulonephropathy in a Young Dog

“…In humans, the clinical features of CFGN are highly variable and can be divided into two general types based on age at onset of symptoms [6]. The first type typically comprises sporadic symptoms and is detected in adulthood by persistent protenuria [8]. The severity of clinical symptoms increases slowly and onset of renal dysfunction is delayed.…”

Glomerulopathy with distinctive fibrillar deposits but lacking glomerular deposition of type III collagen

“…Glomerular accumulation of type I collagen has also been demonstrated in collagenofibrotic glomerulopathy [14,15]. One case of collagenofibrotic glomerulopathy with massive glomerular deposition of both type III and V collagen has been reported [16].…”