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

• Berger Disease
• IgA Glomerulonephritis
• IgAN (IgA Nephropathy)

What is IgA Nephropathy? (Definition/Background Information)

• Berger Disease, also known as IgA Nephropathy (IgAN), is akidney disorderthat occurs when IgA (immunoglobulin A), aproteinthat helps the body fight infections, settles in the kidneys
• IgA Nephropathy can occur at any age, even in childhood. After many years, deposits of IgA may cause the kidneys to leak blood and sometimes protein in the urine 
• In the early stages, IgA Nephropathy has no symptoms. The first sign of this condition may be blood in the urine. After 10 to 20 years, the kidneys may show signs of damage and 20-40% of adults develop end-stage kidney disease
• In most instances, the cause of this condition is unknown; however, certain disorders have been linked with IgA Nephropathy, such ascirrhosis of the liver,celiac disease, andHIV infection 
• Although IgA Nephropathy usually occurs in a family with no other affected members, several cases offamilialIgA Nephropathy have been reported
• Familial IgA Nephropathy is suspected to run through families in anautosomal dominantmanner and is linked to agenetic materialon the long arm ofchromosome 6
• There is no cure for this condition. Treatment focuses on slowing the disease and preventing complications

(Source: IgA Nephropathy; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets IgA Nephropathy? (Age and Sex Distribution)

• IgA Nephropathy is a rare congenital disorder, with symptom manifestation beginning between the ages 15 and 35 years
• Both males and females may be affected, although males are 2-3 times more susceptible than females
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for IgA Nephropathy? (Predisposing Factors)

The following are some risk factors associated with IgA Nephropathy:

• A positive family history, since IgA Nephropathy can be inherited (known as familial IgA Nephropathy)
• Liver cirrhosis
• HIV infection
• Celiac disease

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 IgA Nephropathy? (Etiology)

The exact underlying gene mutation(s) that cause of IgA Nephropathy is currently unknown.

• Researchers have identified that the inherited forms of the disorder is linked to the long arm of chromosome 6
• In such cases, the disorder is inherited in an autosomal dominant manner (i.e., when it runs in a family)

Autosomal dominant: Autosomal dominant conditions are traits or disorders that are present when only one copy of the mutation is inherited on a non-sex chromosome. In these types of conditions, the individual has one normal copy and one mutant copy of the gene. The abnormal gene dominates, masking the effects of the correctly function gene. If an individual has an autosomal dominant condition, the chance of passing the abnormal gene on to their offspring is 50%. Children, who do not inherit the abnormal gene, will not develop the condition or pass it on to their offspring.

What are the Signs and Symptoms of IgA Nephropathy?

The signs and symptoms of IgA Nephropathy may include:

• IgA deposition in the glomerulus
• Proteinuria (protein in urine)
• Nephritis (inflammation of kidneys)
• Purpura (breaking of small blood vessels)
• Hypertension
• Kidney malfunction

How is IgA Nephropathy Diagnosed?

IgA Nephropathy 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 IgA Nephropathy?

The complications of IgA Nephropathy may include:

• Chronic kidney malfunction
• Kidney failure and end-stage renal disease
• Abnormal heart function due to hypertension
• Heart attack

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

How is IgA Nephropathy Treated?

There is no cure for IgA Nephropathy, since it is a progressively deteriorating condition. The treatment is usually given to manage the signs and symptoms and any complication that may develop.

How can IgA Nephropathy be Prevented?

Currently, IgA Nephropathy may not be preventable, since it may have a genetic cause.

• Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
• If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders

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

What is the Prognosis of IgA Nephropathy? (Outcomes/Resolutions)

• The prognosis of IgA Nephropathy is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications
• Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for IgA Nephropathy:

IgA Nephropathy is also known by the following names: 

• Berger's Disease 
• Nephritis, IgA type

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

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