Glomerulonephritis (Nephritic

Syndrome)
by Navin Jaipaul, MD, MHS

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Kidney Filtering Disorders

Overview of Kidney Filtering Disorders

Glomerulonephritis (Nephritic Syndrome)
Asymptomatic Proteinuria and Hematuria Syndrome
Hereditary Nephritis
Acute Tubular Necrosis
Nephrotic Syndrome
Tubulointerstitial Nephritis
Reflux Nephropathy

Glomerulonephritis (nephritic syndrome) is a disorder of

glomeruli (clusters of microscopic blood vessels in the kidneys
with small pores through which blood is filtered). It is
characterized by body tissue swelling (edema), high blood
pressure, and the presence of red blood cells in the urine.

Glomerulonephritis can be caused by various disorders, such as infections, an

inherited genetic disorder, or autoimmune disorders.

Diagnosis is based on tests of blood and urine and sometimes imaging tests, a
biopsy of the kidneys, or both.

People often need to restrict salt and protein intake and take diuretics or
antibiotics until kidney function improves.

Glomerulonephritis can develop over a short time period (acute glomerulonephritis) or

develop and progress slowly (chronic glomerulonephritis). In 1% of children and 10% of
adults who have acute glomerulonephritis, it evolves into rapidly progressive
glomerulonephritis, in which most of the glomeruli are destroyed, resulting in kidney
failure.

Causes
Glomerulonephritis can be primary, originating in the kidneys, or secondary,
caused by a vast array of disorders. Those disorders may affect other parts of
the body.

Acute glomerulonephritis
Acute glomerulonephritis most often occurs as a complication of a throat or
skin infection with streptococcus, a type of bacteria. Acute glomerulonephritis
that occurs after a streptococcal infection (poststreptococcal
glomerulonephritis) typically develops in children between the ages of 2 and
10 after recovery from the infection. Infections with other types of bacteria,
such as staphylococcus and pneumococcus, viral infections, such as
chickenpox, and parasitic infections, such as malaria, can also result in acute
glomerulonephritis. Acute glomerulonephritis that results from any of these
infections is called postinfectious glomerulonephritis.
Acute glomerulonephritis may also be caused by noninfectious disorders,
including membranoproliferative glomerulonephritis, immunoglobulin A
(IgA) nephropathy, immunoglobulin Aassociated vasculitis (formerly,
Henoch-Schnlein purpura), systemic lupus erythematosus (lupus),
cryoglobulinemia, Goodpasture syndrome, and granulomatosis with
polyangiitis (formerly, Wegener granulomatosis). Acute glomerulonephritis
that develops into rapidly progressive glomerulonephritis most often results
from conditions that involve an abnormal immune reaction.

Chronic glomerulonephritis

Often, chronic glomerulonephritis seems to result from some of the same

conditions that cause acute glomerulonephritis, such as IgA nephropathy or
membranoproliferative glomerulonephritis. Sometimes, acute
glomerulonephritis does not resolve and instead becomes long lasting
(chronic). Occasionally, chronic glomerulonephritis is caused by hereditary
nephritis, an inherited genetic disorder. In many people, the cause of chronic
glomerulonephritis cannot be identified.
SOME CAUSES OF GLOMERULONEPHRITIS
Infections
o

Bacterial infections (for example, with streptococcus, staphylococcus, or

pneumococcus)

Fungal infections

Parasitic infections (for example, malaria)

Viral infections (for example, hepatitis B and C or HIV infections)

Vasculitis (blood vessel inflammation)

Cryoglobulinemia

Eosinophilic granulomatosis with polyangiitis (formerly, Churg-Strauss

syndrome)

Granulomatosis with polyangiitis (formerly, Wegener granulomatosis)

Microscopic polyangiitis
Immune disorders

Goodpasture syndrome

Systemic lupus erythematosus (lupus)

Other causes

Hereditary nephritis

Drugs (for example, quinine, gemcitabine, or mitomycin C)

Symptoms
About half of the people with acute glomerulonephritis have no
symptoms. If symptoms do occur, the first to appear are tissue swelling
(edema) due to fluid retention, low urine volume, and production of urine that

is dark because it contains blood. Edema may first appear as puffiness of the
face and eyelids but later is prominent in the legs. Blood pressure increases as
kidney function becomes impaired. Some people become drowsy or confused.
In older people, nonspecific symptoms, such as nausea and a general feeling of
illness (malaise), are more common.
When rapidly progressive glomerulonephritis develops, weakness,
fatigue, and fever are the most frequent early symptoms. Loss of appetite,
nausea, vomiting, abdominal pain, and joint pain are also common. About
50% of people have a flu-like illness in the month before kidney failure
develops. These people have edema and usually produce very little urine. High
blood pressure is uncommon and rarely severe when it does occur.
Because chronic glomerulonephritis usually causes only very mild or
subtle symptoms, it goes undetected for a long time in most people. Edema
may occur. High blood pressure is common. The disease may progress to
kidney failure, which can cause itchiness, decreased appetite, nausea,
vomiting, fatigue, and difficulty breathing.

Diagnosis
Doctors investigate the possibility of acute glomerulonephritis in people
whose laboratory test results indicate kidney dysfunction or blood in the urine
and in people who develop symptoms of the disorder. Laboratory tests show
variable amounts of protein and blood cells in the urine and often kidney
dysfunction, as shown by a high concentration of urea and creatinine (waste
products) in the blood.
In people with rapidly progressive glomerulonephritis, casts (clumps of red
blood cells or white blood cells) are often visible in a urine sample that is
examined under a microscope. Blood tests usually detect anemia.
When doctors suspect glomerulonephritis, a biopsy of a kidney is usually done
to confirm the diagnosis, help determine the cause, and determine the amount
of scarring and potential for reversibility. Kidney biopsy is done by inserting a
needle in one of the kidneys under ultrasound or computed tomography (CT)

guidance to obtain a small amount of kidney tissue. Although kidney biopsy is

an invasive procedure and occasionally can become complicated, it is usually
safe.
Additional tests are sometimes helpful for identifying the cause. For example,
in the diagnosis of postinfectious glomerulonephritis, a throat culture may
provide evidence of streptococcal infection. Blood levels of antibodies against
streptococci may be higher than normal or progressively increase over several
weeks. Acute glomerulonephritis that follows an infection other than strep
throat is usually easier to diagnose because its symptoms often begin while the
infection is still obvious. Cultures and blood tests that help identify the
organisms that cause these other types of infections are sometimes needed to
confirm the diagnosis.
When doctors suspect an autoimmune cause for glomerulonephritis, they do
blood tests for antibodies directed against some of the body's own tissues
(called autoantibodies) and tests that assess the complement system, a system
of proteins involved in the body's immune system.
Chronic glomerulonephritis develops gradually, and therefore, a doctor may
not be able to tell exactly when it began. It may be discovered when a urine
test, done as part of a medical examination, reveals the presence of protein
and blood cells in the urine in a person who is feeling well, has normal kidney
function, and has no symptoms. Doctors usually do an imaging test of the
kidneys, such as ultrasonography or CT. A kidney biopsy is the most reliable
way to distinguish chronic glomerulonephritis from other kidney disorders. A
biopsy, however, is rarely done in advanced stages. In these cases, the kidneys
are shrunken and scarred, and the chance of obtaining specific information
about the cause is small. Doctors suspect that the kidneys are shrunken and
scarred if kidney function has been poor for a long time and the kidneys
appear abnormally small on an imaging test.

Prognosis

Acute poststreptococcal glomerulonephritis resolves completely in most cases,

especially in children. About 1% of children and 10% of adults develop chronic
kidney disease.
The prognosis for people with rapidly progressive glomerulonephritis depends
on the severity of glomerular scarring and whether the underlying disease,
such as infection, can be cured. In some people who are treated early (within
days to weeks), kidney function is preserved and dialysis is not needed.
However, because the early symptoms can be subtle and vague, most people
who have rapidly progressive glomerulonephritis are not aware of the
underlying disease and do not seek medical care until kidney failure develops.
If treatment occurs late, the person is more likely to develop chronic kidney
disease with kidney failure. Because kidney failure tends to develop before
people notice it, 80 to 90% of people who have rapidly progressive
glomerulonephritis become dependent upon dialysis. The prognosis also
depends on the cause, the person's age, and any other diseases the person
might have. When the cause is unknown or the person is older, the prognosis
is worse.
In some children and adults who do not recover completely from acute
glomerulonephritis, other types of kidney disorders develop, such as
asymptomatic proteinuria and hematuria syndrome or nephrotic syndrome.
Other people with acute glomerulonephritis, especially older adults, often
develop chronic glomerulonephritis.
Primary Glomerular Disorders That Can Cause
Glomerulonephritis

Disorder

Description

Prognosis

Fibrillary

In this rare disease, abnormal proteins are

The prognosis is poor. End-stage kidney failure occurs

glomerulonephritis

deposited around the glomerulus. It may also

in half of people within 4 years. It is not clear whether

cause nephrotic syndrome.

treatment (with corticosteroids and

immunosuppressants) helps.

Disorder

Description

Prognosis

Primary rapidly

This group of disorders causes microscopic

The prognosis is poor. At least 80% of people who are

progressive

damage to the glomeruli and progress

not treated develop end-stage kidney failure within 6

glomerulonephritis

rapidly. Sometimes they are caused by an

months. The prognosis is better for people younger than

infection or other treatable disorder.

60 years and when an underlying disorder causing the

glomerulonephritis responds to treatment. With
treatment, 43% of people develop end-stage renal
disease within 12 months.

Immunoglobulin A (IgA)

The most common form of

Usually the disorder progresses slowly. End-stage

nephropathy

glomerulonephritis in the world is caused by

kidney failure develops in about 25% of people after 20

immune complexes (combinations of

years. The disorder progresses more slowly in children.

antigens and antibodies) deposited in the

kidneys.

Membranoproliferative

This uncommon type of glomerulonephritis

If the cause is known and can be treated, a partial

glomerulonephritis

occurs primarily between the ages of 8 and

remission may occur. The outcome is not as good in

30. The disorder is caused by immune

people in whom the cause remains unknown. About half

complexes (combinations of antigens and

of people progress to end-stage kidney failure within 10

antibodies) attaching to the kidneys, but

years, and 90% within 20 years.

sometimes why the complexes attach to the

kidneys is unknown.

Treatment
No specific treatment is available in most cases of acute glomerulonephritis.
The disorder causing glomerulonephritis is treated when possible. Following a
diet that is low in protein and sodium may be necessary until kidney function
recovers. Diuretics may be prescribed to help the kidneys excrete excess
sodium and water. High blood pressure needs to be treated.

When a bacterial infection is suspected as the cause of acute

glomerulonephritis, antibiotics are usually ineffective because the nephritis
begins 1 to 6 weeks (average, 2 weeks) after the infection, which has, by then,
usually resolved. However, if a bacterial infection is still present when acute
glomerulonephritis is discovered, antibiotic therapy is started. Antimalarial
drugs may be beneficial if glomerulonephritis is caused by malaria. Some
autoimmune disorders that cause glomerulonephritis are treated with
corticosteroids, drugs that suppress the immune system, or both.
For rapidly progressive glomerulonephritis, drugs to suppress the immune
system are started promptly. High doses of corticosteroids are usually given
intravenously for about a week, followed by a variable period of time when
they are taken by mouth. Cyclophosphamide, an immunosuppressant, may
also be given. In addition, plasma exchange is sometimes used to remove
antibodies from the blood. The sooner treatment occurs, the less likely are
kidney failure and the need for dialysis. Kidney transplantation is sometimes
considered for people who develop chronic kidney disease with kidney failure,
but rapidly progressive glomerulonephritis may recur in the transplanted
kidney.
Taking either an angiotensin-converting enzyme (ACE) inhibitor or an
angiotensin II receptor blocker (ARB) often slows progression of chronic
glomerulonephritis and tends to reduce blood pressure and the excretion of
protein in the urine. Reducing blood pressure and sodium intake are
considered beneficial. Restricting the amount of protein in the diet is modestly
helpful in reducing the rate of kidney deterioration. End-stage kidney failure
can be treated with dialysis or a kidney transplant.