What are the other Names for this Condition? (Also known as/Synonyms)

• Glomerulonephritis with Organized Monoclonal Microtubular Immunoglobulin Deposits (GOMMID)
• GOMMID (Glomerulonephritis with Organized Monoclonal Microtubular Immunoglobulin Deposits)
• Immunotactoid Glomerulonephritis

What is Immunotactoid Glomerulopathy? (Definition/Background Information)

• Immunotactoid Glomerulopathy, also known as Glomerulonephritis with Organized Monoclonal Microtubular Immunoglobulin Deposits (GOMMID), is a very uncommon cause of glomerular disease. The disorder probably results 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
• Immunotactoid Glomerulopathy has 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

(Source: Immunotactoid Glomerulopathy; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Who gets Immunotactoid Glomerulopathy? (Age and Sex Distribution)

• Immunotactoid Glomerulopathy is a very rare disorder. The presentation of symptoms most often occurs in adulthood
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Immunotactoid Glomerulopathy? (Predisposing Factors)

The risk factors for Immunotactoid Glomerulopathy may include the following:

• Autoimmune disorders
• Hepatitis C infection
• Leukocytoclastic vasculitis
• Hypocomplementemia
• A malignant cancer

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Immunotactoid Glomerulopathy? (Etiology)

The exact cause of Immunotactoid Glomerulopathy is not known at the present time. The condition may occur spontaneously, or may be associated with the following conditions:

• Hepatitis C infection
• Cancers
• Autoimmune disorders
• Leukocytoclastic vasculitis
• Hypocomplementemia

What are the Signs and Symptoms of Immunotactoid Glomerulopathy?

The signs and symptoms of Immunotactoid Glomerulopathy may include the following:

• Blood in urine
• Protein in urine
• Kidney malfunction
• High blood pressure
• Ascites
• Edema
• Pleural effusion

How is Immunotactoid Glomerulopathy Diagnosed?

Immunotactoid Glomerulopathy is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Immunotactoid Glomerulopathy?

The complications of Immunotactoid Glomerulopathy may include:

• Frequent infections
• Increased risk of blood clots
• Increased risk for developing chronic lymphocytic leukemia and B cell lymphomas
• Chronic kidney failure leading to end-stage renal disease

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Immunotactoid Glomerulopathy Treated?

The treatment for Immunotactoid Glomerulopathy is generally geared toward offering relief from signs and symptoms and may include the following:

• Treatment for an underlying disorder that may have caused the condition
• Dietary restrictions to prevent fluid retention and salt accumulation
• Cyclophosphamide 
• Steroids
• Angiotensin-converting enzyme inhibitors (ACE inhibitors) 
• Blood thinners
• Statins
• Dialysis
• Kidney transplantation

How can Immunotactoid Glomerulopathy be Prevented?

At the present time, the exact cause of Immunotactoid Glomerulopathy is not known, and therefore, no guidelines for its prevention are available.

• However, seeking medical attention for certain predisposing conditions, such as an autoimmune disorder or hepatitis C infection, may help minimize the likelihood of developing Immunotactoid Glomerulopathy
• Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as Immunotactoid Glomerulopathy

Regular medical screening at periodic intervals with tests and physical examinations are recommended.

What is the Prognosis of Immunotactoid Glomerulopathy? (Outcomes/Resolutions)

• Immunotactoid Glomerulopathy is a slowly progressive disorder with a poor prognosis. The condition leads to the development of end-stage renal disease in 2-4 years, in about 50% of those affected
• The prognosis is considered to be poorer, if there is extensive glomerular involvement or systemic disease
• In addition, in some cases, Immunotactoid Glomerulopathy is reported to develop in transplanted kidneys
• Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Immunotactoid Glomerulopathy:

• Immunotactoid Glomerulopathy is related to a similar disease known as fibrillary glomerulopathy, which is a more common disorder

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/