Immunotactoid or fibrillary glomerulopathy is a term that includes two conditions:
immunotactoid glomerulopathy and
fibrillary glomerulonephritis, which are uncommon causes of
glomerular disease. Most experts feel that fibrillary glomerulonephritis and immunotactoid glomerulopathy are separate disorders but they have many similarities and some experts group these disorders together. Fibrillary glomerulonephritis and immunotactoid glomerulopathy can be distinguished from each other by
electron microscopy; the 'fibrils' that characterize fibrillary glomerulonephritis are smaller and randomly oriented as opposed to the larger and organized fibrils of immunotactoid glomerulopathy which also have
microtubule formations. Both disorders probably result from deposits derived from
immunoglobulins but in most cases the cause is idiopathic (unknown). The signs and symptoms are similar in both diseases and may include blood (hematuria) and
protein (
proteinuria) in the urine, kidney insufficiency and
high blood pressure. Fibrillary glomeurlonephritis is much more common than immunotactoid glomerulopathy. Both fibrillary glomerulonephritis and immunotactoid glomerulopathy have been associated with
hepatitis C virus infection and with malignancy and
autoimmune disease, but immunotactoid glomerulopathy patients have a greater predisposition to
chronic lymphocytic leukemia and
B cell lymphomas. All patients should be screened for these conditions. It is also important to rule out another disease known as
amyloidosis. When the fibrils are stained with an acid dye known as "Congo red" the results are negative. In amyloidosis the results are positive because the dye is absorbed by the amyloids. Treatment is generally determined by the severity of the kidney problems.
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Last updated: 8/18/2015
