Fibrillary glomerulonephritis

From WikiMD's Wellness Encyclopedia

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.


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