National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Immunotactoid glomerulopathy



Other Names:
Immunotactoid glomerulonephritis
Categories:
This disease is grouped under:

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). 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). The signs and symptoms include blood (hematuria) and protein (proteinuria) in the urine, kidney insufficiency and high blood pressure. 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. Treatment is generally determined by the severity of the kidney problems.[1]
Last updated: 8/18/2015

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.[2][1]
Last updated: 3/28/2014

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnosis of ITG includes simple cryoglobulinemia, systemic lupus erythematosus, amyloidosis and non-amyloid FGP (see these terms).
Visit the Orphanet disease page for more information.

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.

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  • If you have a kidney transplant from a living donor, what are the chances of this disease attacking the new kidney? What is the long-term prognosis of this disease? I can find very little information. See answer



  1. Fervenza F, Sethi S, Appel G. Glomerular diseases due to nonamyloid fibrillar deposits. UpToDate. July 16, 2014; http://www.uptodate.com/contents/glomerular-diseases-due-to-nonamyloid-fibrillar-deposits. Accessed 8/19/2015.
  2. Immunotactoid Glomerulopathy. UNC Health Care Kidney Center. http://www.unckidneycenter.org/kidneyhealthlibrary/immunotactoid.html. Accessed 3/28/2014.