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

• Fibrillary Glomerulonephritis and Immunotactoid Glomerulopathy
• Immunotactoid or Fibrillary Glomerulonephritis
• Immunotactoid or Fibrillary Glomerulopathy

What is Immunotactoid or Fibrillary Glomerulopathy? (Definition/Background Information)

• Immunotactoid or Fibrillary Glomerulopathy is a term that includes two conditions: Immunotactoid Glomerulopathy and Fibrillary Glomerulonephritis, which are uncommon causes of glomerular disease
• Most experts feel that fibrillary glomerulonephritis and immunotactoid glomerulopathy are separate disorders but they have many similarities and some experts group these disorders together
• Fibrillary glomerulonephritis and immunotactoid glomerulopathy can be distinguished from each other by electron microscopy; the 'fibrils' that characterize fibrillary glomerulonephritis are smaller andrandomly oriented as opposed to the larger and organized fibrils of immunotactoid glomerulopathy which also have microtubule formations
• Both disorders probably result from deposits derived from immunoglobulins but in most cases the cause is idiopathic (unknown)
• The signs and symptoms are similar in both diseases and may 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, but immunotactoid glomerulopathy patients have a greater predisposition to chronic lymphocytic leukemia and B cell lymphomas
• Treatment is generally determined by the severity of the kidney problems

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

Who gets Immunotactoid or Fibrillary Glomerulopathy? (Age and Sex Distribution)

• Immunotactoid or Fibrillary Glomerulopathy is a rare disorder. Of the two disorders, fibrillary glomerulopathy is 10 times more common than the immunoactoid form
• The presentation of symptoms typically 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 or Fibrillary Glomerulopathy? (Predisposing Factors)

The risk factors for Immunotactoid or Fibrillary Glomerulopathy may include:

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

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 or Fibrillary Glomerulopathy? (Etiology)

The cause of Immunotactoid or Fibrillary Glomerulopathy is not known clearly. 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 or Fibrillary Glomerulopathy?

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

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

How is Immunotactoid or Fibrillary Glomerulopathy Diagnosed?

Immunotactoid or Fibrillary 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
• Differential diagnosis to rule out amyloidosis: Staining the fibrils with "Congo red" yields a negative result for Immunotactoid or Fibrillary Glomerulopathy. On the other hand, the dye is absorbed by the amyloid deposits in amyloidosis, and the results are positive 

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 or Fibrillary Glomerulopathy?

The complications of Immunotactoid or Fibrillary Glomerulopathy may include:

• Frequent infections
• Increased risk of blood clots
• Increased risk for developing chronic lymphocytic leukemia and B cell lymphomas (specifically in those affected by the immunotactoid glomerulopathy)
• 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 or Fibrillary Glomerulopathy Treated?

The treatment for Immunotactoid or Fibrillary 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 or Fibrillary Glomerulopathy be Prevented?

At the present time, the exact cause of Immunotactoid or Fibrillary Glomerulopathy is not known, and no preventive methods or guidelines are available at the present time.

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

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

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

• The prognosis of Immunotactoid or Fibrillary Glomerulopathy is dependent upon the severity of the signs and symptoms and associated complications, if any
• The disorder is slowly progressive, with end-stage kidney disease developing in many 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 or Fibrillary Glomerulopathy:

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

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