Fibrillary glomerulonephritis
Other Names: Non-amyloid fibrillary glomerulopathy; Congo red-negative amyloidosis-like glomerulopathy; Non-amyloid fibrillary glomerulonephritis
Fibrillary glomerulonephritis is an uncommon cause of glomerular disease. A more rare disorder known as immunotactoid glomerulpathy is a very similar condition. Both disorders probably result from deposits derived from immunoglobulins but in most cases the cause is idiopathic (unknown).
Cause[edit | edit source]
Both fibrillary glomerulonephritis and immunotactoid glomerulopathy have been associated with hepatitis C virus infection and with malignancy and autoimmune disease. Because of this, patients should be screened for these conditions.
Signs and symptoms[edit | edit source]
The signs and symptoms include blood (hematuria) and protein (proteinuria) in the urine, kidney insufficiency and high blood pressure.
Diagnosis[edit | edit source]
The diagnosis is made with a kidney biopsy and by electron microscopy. Fibrillary glomerulonephritis and immunotactoid glomerulopathy can be differentiated from each other by electron microscopy; the fibrils in fibrillary glomerulonephritis are smaller and randomly oriented as opposed to the larger and often organized fibrils of immunotactoid glomerulopathy.
Treatment[edit | edit source]
Treatment is generally determined by the severity of the kidney problems. The most common form of treatment was steroids, with or without a second agent, including most notably cyclophosphamide or rituximab. A benefit of immunosuppression could not be detected in these small, retrospective studies in which patients with more severe disease were more likely to have been treated.
Prognosis[edit | edit source]
The prognosis for patients with FGN remains poor, with limited data to suggest optimal therapy.
NIH genetic and rare disease info[edit source]
Fibrillary glomerulonephritis is a rare disease.
Fibrillary glomerulonephritis Resources | |
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