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Glomerular diseases due to nonamyloid fibrillar deposits

Fernando C Fervenza, MD, PhD
Sanjeev Sethi, MD, PhD
Section Editors
Richard J Glassock, MD, MACP
Brad H Rovin, MD
Deputy Editor
John P Forman, MD, MSc


Glomerular disease may be associated with the appearance of fibrillar deposits in the mesangium or glomerular basement membrane. The fibrils are Congo red-positive in amyloidosis and Congo red-negative in other forms. The most common forms of Congo red-negative (nonamyloid) fibrillar glomerular deposition diseases are fibrillary glomerulonephritis and immunotactoid glomerulopathy.

Fibrillary glomerulonephritis [1-6] and immunotactoid glomerulopathy [3,5-9] are uncommon causes of glomerular disease. Both disorders probably result from deposits derived from immunoglobulins [9]. The pathognomonic histologic findings are seen on electron microscopy. By comparison, other forms of nonamyloid fibrillary deposition disease have been described in which the deposits are composed of fibronectin (fibronectin glomerulopathy) or atypical type III collagen fibrils (collagenofibrotic glomerulopathy). (See 'Pathology and pathogenesis' below and 'Other fibrillary disorders' below.)

Glomerular disease associated with amyloid deposits is discussed separately. (See "Renal amyloidosis".)


Fibrillary glomerulonephritis and immunotactoid glomerulopathy are uncommon disorders, being present in 0.5 to 1 percent of native renal biopsies [3,6]. Most investigators feel that, based upon histologic features and clinical associations, fibrillary glomerulonephritis and immunotactoid glomerulopathy are separate disorders, with fibrillary glomerulonephritis accounting for approximately 85 to 90 percent of cases [3,5,8]. However, some believe that they are pathogenetically related, with no compelling histologic or clinical reasons to separate the two. (See 'One or two disorders?' below.)

Pathology and pathogenesis

Fibrillary glomerulonephritis — The diagnosis of fibrillary glomerulonephritis is established by renal biopsy, with the pathognomonic changes seen on electron microscopy.


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Literature review current through: Sep 2016. | This topic last updated: Jul 16, 2014.
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