Primary Glomerulonephritis

Alternate Names
GN

Definition of Primary Glomerulonephritis

Primary glomerulonephritis is responsible for an inflammation of the glomeruli or filtering portions of the kidneys. When glomeruli are damaged, protein and blood cells may leak into the urine and the kidney may lose function completely over time. There are many different types of primary GN.

What is going on in the body?

Primary GN leads to damage of the glomeruli. This is an inflammation as a result of immune cells and antibodies depositing in the kidney's blood filters called glomeruli. The injury can be mild with few symptoms, can come and go, or be chronic and permanent. It can be severe and life threatening.

Major syndromes associated with primary GN, include:

• acute glomerulonephritis, which may develop suddenly and may result from a previous Group A streptococcus infection of the throat ("strep throat") or skin (impetigo or Scarlet Fever)
• rapidly progressive glomerulonephritis (called RPGN) which has numerous causes,
• a few other diseases such as Berger's, Henoch-Schonlein, vasculitis and lupus as well as similar "autoimmune" (or directed against self) inflammations.
• diabetes mellitus
• chronic glomerulonephritis, which may develop slowly or be the result of a severe initial inflammation that progresses later.
• nephrotic syndrome, which causes a set of symptoms including body swelling called "edema" and an elevated cholesterol in the blood
• nephritic syndrome or blood in the urine and high blood pressure often with kidney function loss

What are the signs and symptoms of the disease?

Following are some of the symptoms of GN:

• blood in the urine
• decreased or increased urine output
• high blood pressure
• shortness of breath
• swelling, especially in the hands, feet, ankles, and face (around the eyes)
• loss of kidney function

Primary GN can also cause uremia. This is a high level of waste products in the blood that requires dialysis to relieve it. The dialysis can be a permanent requirement.

Symptoms of uremia include:

• cognitive impairments, or changes in mental status
• fatigue
• hiccups
• nausea and vomiting
• electrolyte or salt imbalances
• heart failure from fluid retention
• inflammation around the heart called pericarditis

What are the causes and risks of the disease?

Several factors can contribute to the risk for primary GN. These include the following:

• diabetes mellitus is a major cause of GN
• autoimmune disorders, such as a condition called systemic lupus erythematosus
• certain cancers
• hepatitis from hepatitis B and C infections that result from drug use or unsafe sex
• genetic tendencies (a disease called Alport Syndrome, for example)
• infections, such as a heart infection known as endocarditis
• Group A streptococcal infection such as strep throat or skin infections.
• the HIV virus can cause glomerular disease
• diseases associated with a particular type of antibody called an ANCA can cause GN
• vasculitis can cause GN

What can be done to prevent the disease?

Treatment of the underlying disease (like lupus) or infection (like hepatitis B and/or C) may prevent or help some cases of GN.

Avoiding high risk activities (drugs, unsafe sex) is essential.

Unfortunately many cases of GN are "idiopathic" meaning the cause is unknown and therefore cannot be avoided.

How is the disease diagnosed?

Diagnosis of GN begins with a medical history and physical exam. The healthcare provider may also order urine (especially an initial urinalysis) and blood tests.

Blood tests useful in making the diagnosis include:

• antibody titer tests that may detect an autoimmune disorder like an "ANA" for lupus)
• antibody titer tests that may detect an infection such as chronic hepatitis B or C.
• protein tests that determine how much protein there is in the urine.
• a complement level that is decreased with some GNs (after a strep infection, with lupus)
• ANCA test

Other tests include tests for certain malignancies (myeloma, colon or kidney cancer).

Also, a renal biopsy is usually needed to confirm the diagnosis. The biopsy involves using a special needle to obtain a piece of tissue from the kidney. The tissue is then examined under a microscope.

What are the long-term effects of the condition?

If the GN is treatable, as a result of Hepatitis C or lupus, treatment is instituted, but does not always arrest the disease.

Many of the GNs are not treatable (membrano-proliferative or focal GS are examples) and they may progress to complete kidney failure.

Other long-term effects depend on the underlying cause. For example, certain GNs (like that due to a disease called Wegeners) also affect the lung which can be seriously damaged.

What are the risks to others?

Glomerulonephritis is not contagious and poses no risk to others. If an infection, such as hepatitis, causes it, the infection may be contagious.

What are the treatments for the disease?

Treatments for primary GN are as follows:

• tight control of diabetes if it is te cause of GN
• ACE inhibitors (ACEIs), such as enalapril (i.e., Vasotec), lisinopril (i.e., Prinivil, Zestril), or captopril (i.e., Capoten)
• ARBS another class of BP medicine may be used alone or with ACEIs because they not only lower blood pressure but also decrease protein in the urine.
• antibiotics if an infection is present (like with strep)
• corticosteroids, such as prednisone or other intravenous kinds
• cytotoxic agents, which inhibit the immune system (like cyclophosphamide <i.e., Cytoxan> or azathioprine <i.e., Imuran>)
• diuretics or water pills to decrease fluid retention/edema
• medicines that lower cholesterol may be used, such as the statins.
• hemodialysis (temporary or permanent) by hooking the person with GN or kidney failure up to a machine that filters the blood
• chemotherapy for some cancers (like myeloma)
• kidney transplant
• a special diet for kidney disease (limiting protein and potassium)

What are the side effects of the treatments?

Long-term use of corticosteroids can cause osteoporosis or a softening of the bones.. Immunosuppressive and cytotoxic therapies can increase a person's risk of infections and cancer.

What happens after treatment for the disease?

Expectations for recovery vary according to the type and extent of the disease when treatment starts. If the cause is reversible and is treated, no further treatment may be required. A person can often return to normal activities. Treatment may be prolonged if the underlying cause cannot be fixed. Some individuals may develop chronic glomerulonephritis or kidney failure.

How is the disease monitored?

Someone who has primary GN will have regular visits with the healthcare provider. Blood pressure and kidney function will be monitored as well as other blood tests mentioned earlier. Any new or worsening symptoms should be reported to the healthcare provider.

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