Immunotactoid glomerulopathy

Other Names: Immunotactoid glomerulonephritis

Immunotactoid glomerulopathy, also known as glomerulonephritis with organized monoclonal microtubular immunoglobulin deposits (GOMMID), is a very uncommon cause of glomerular disease. It is related to a similar disease known as fibrillary glomerulopathy, which is more common. Both disorders probably result from deposits derived from immunoglobulins, but in most cases the cause is idiopathic (unknown). Both fibrillary glomerulonephritis and immunotactoid glomerulopathy have been associated with hepatitis C virus infection and with malignancy and autoimmune disease. Also, patients with immunotactoid glomerulopathy have a greater risk to have chronic lymphocytic leukemia and B cell lymphomas and should be screened for all of these conditions.

Signs and symptoms

The signs and symptoms include blood (hematuria) and protein (proteinuria) in the urine, kidney insufficiency and high blood pressure.

Diagnosis

On electron microscopy, immunotactoid glomerulopathy is characterized by the formation of microtubules which are much larger than the fibrils observed in fibrillary glomerulonephritis (30 to 50 versus 16 to 24 nm in diameter).

Treatment

Treatment is generally determined by the severity of the kidney problems.

Prognosis

The long-term prognosis for immunotactoid glomerulopathy is difficult to predict due to the rarity of the condition, but some studies suggest that 50% of patients will have end-stage kidney disease (ESKD) by 5 years after diagnosis. Patients with ESKD may either pursue dialysis or kidney transplantation. The disease may recur in the transplanted kidney. However, the degree and rate of recurrence remains unclear due to the rarity of this disease. Researchers believe the rate of progression of the disease in the transplanted kidney is slower than in the native kidney. The rate of recurrence seems to be higher in patients who have monoclonal gammopathy.

NIH genetic and rare disease info

• NIH info on Immunotactoid glomerulopathy

Immunotactoid glomerulopathy is a rare disease.

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