GLOMERULAR SYNDROMES

LIGIA PETRESCU

BACKGROUND
This class of kidney disease centers around the
glomerulus.
This is where the main filtration of the
nephron occurs and is located within the
Bowman's capsule.
It is comprised of a mass of tiny tubes through
which the blood passes.
Its semipermeable structure allows water and
soluble wastes to pass through and be
excreted out of the Bowman's capsule as urine.
The filtered blood passes out of the
glomerulus into the efferent arteriole to be
returned through the medullary plexus to the
intralobular vein.
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CLASIFICATION

ACUTE NEPHRITIC SYNDROME

RAPIDLY PROGRESSIVE
GLOMERULONEPHRITIS
NEPHROTIC SYNDROME
ASYMPTOMATIC HEMATURIA OR
PROTEINURIA
CHRONIC GLOMERULONEPHRITIS
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ACUTE NEPHRITIC SYNDROME

DEFINITION AND CLASIFICATION
Acute nephritic syndrome is a group of disorders that cause
inflammation of the internal kidney structures (specifically,
the glomeruli), often caused by an immune response
triggered by an infection or other disease.
It is characterized by tissue swelling (edema), high blood
pressure, the presence of red blood cells in the urine, red
cell casts, proteinuria, renal failure.
Nephritic syndrome can develop suddenly or over a short
time period (acute nephritic syndrome) or develop and
progress slowly (chronic nephritic syndrome).
In 1% of children and 10% of adults, the acute nephritic
syndrome evolves into rapidly progressive
glomerulonephritis, in which most of the glomeruli are
destroyed, resulting in kidney failure
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ETIOLOGY
The cause of chronic nephritic syndrome
cannot be identified in many people.
Often, chronic nephritic syndrome seems to
result from one of the same conditions that
causes acute nephritic syndrome.

PRIMARY GLOMERULOPATHIES

ACUTE DIFFUSE PROLIFERATIVE

GLOMERULONEPHRITIS
RAPIDLY PROGRESSIVE (CRESCENTIC)
GLOMERULONEPHRITIS
MEMBRANOUS GLOMERULOPATHY
MINIMAL CHANGE DISEASE
FOCAL SEGMENTAL GLOMERULOSCLEROSIS
MEMBRANOPROLIFERATIVE
GLOMERULONEPHRITIS
IgA NEPHROPATHY
FOCAL PROLIFERATIVE GLOMERULONEPHRITIS
FIBRILLARY GLOMERULOPATHY
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SYSTEMIC DISEASES

SYSTEMIC LUPUS ERYTHEMATOSUS

DIABETES MELLITUS
AMYLOIDOSIS
GOODPASTURE SYNDROME
POLYARTERITIS NODOSA
WEGENER GRANULOMATOSIS
HENOCH-SCHONLEIN PURPURA
BACTERIAL ENDOCARDITIS

INFECTIOUS DISEASES

Bacterial infections

post-streptococcal glomerulonephritis typically

develops following a throat or skin infection in
children between the ages of 2 and 14.

Infections by other types of bacteria, such

as staphylococcus and pneumococcus

viral infections such as chickenpox, hepatitis C,

B, HIV
parasitic infections such as malaria.
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HEREDITARY DISEASES
ALPORT SYNDROME
THIN MEMBRANE DISEASE
FABRY DISEASE

OTHERS
Primary gravidic nephropathy
Mixedema
Dermatological diseases
Obesity

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PATHOGENESIS
ANTIBODY MEDIATED INJURY: Goodpasture antigen
(anti-GBM nephritis)
IN-SITU IMMUNE COMPLEX DEPOSITION
Fixed intrinsic tissue antigens:
Heymann antigen (membranous glomerulonephritis)
Mesangial antigens
Others
Planted antigens:
Exogenous (infections, drugs)
Endogenous (DNA, immunoglobulins, immune
complexes, IgA)

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CIRCULATING IMMUNE COMPLEX

DEPOSITION

CIRCULATING IMMUNE COMPLEX

DEPOSITION
Endogenous antigens (DNA, tumor antigens)
Exogenous antigens (infectious products)
CELL MEDIATED IMMUNE INJURY
Rejet nephropathy

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SYMPTOMS
Despite the diversity of diseases that cause acute
nephritis below, they share many symptoms in
common.
About half of the people with acute nephritic
syndrome have no symptoms.
If symptoms do occur, the first to appear are fluid
retention and tissue swelling (edema), low urine
volume, and dark urine that contains blood.
Edema may first appear as puffiness of the face and
eyelids but later is prominent in the legs.

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SYMPTOMS
Blood pressure increases as kidney function becomes
impaired. In turn, high blood pressure and swelling
of the brain may produce headaches, visual
disturbances, and more serious disturbances of brain
function.
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
obvious early symptoms. Loss of appetite, nausea,
vomiting, abdominal pain, and joint pain are also
common.
About 50% of people had a flu-like illness in the
month before kidney failure started to develop.
These people have edema and usually produce very
little urine. High blood pressure is uncommon and
rarely severe when it does occur.

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SYMPTOMS

Because chronic nephritic syndrome usually causes

only very mild or subtle symptoms for years, it goes
undetected in most people.
Fluid retention (edema) may occur.
High blood pressure is common.
The disease may progress to kidney failure, which can
cause itchiness, fatigue, decreased appetite, nausea,
vomiting, and difficulty breathing.

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SIGNS AND TESTS

The blood pressure may be elevated.
There may be signs of fluid overload (more fluid in
circulation than the heart can effectively pump),
including abnormal heart and lung sounds. The
jugular (neck) veins may be distended from increased
pressure.
Generalized swelling is often present. When
examining the abdomen, may be signs of fluid
overload and an enlarged liver.

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SIGNS AND TESTS

There may be signs of acute kidney failure in

addition to the above symptoms.
Urine appearance and color are abnormal (cocacola).
Urinalysis nephritic urinary sediment reveals
variable number of red blood cells (RBCs), WBCs,
and RBC casts (pathognomonic of active
glomerulonephritis)
Protein in the urine test is positive; Proteinuria is
usually modest, ranging from 2 to 6 g in a 24-hour
collection
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SIGNS AND TESTS

Azotemia (nitrogen-containing waste products in the

blood) may be evidenced by:
elevated BUN
elevated creatinine

potassium test may be elevated.

The creatinine clearance may be decreased.
kidney biopsy reveals glomerulonephritis
(inflammation of the glomeruli), confirm the
diagnosis, help determine the cause, and determine
the amount of scarring and potential for reversibility.
A biopsy, however, is rarely performed in advanced
stages, when the kidneys are shrunken and scarred,
because the chance of obtaining specific information
about the cause is small.

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SIGNS AND TESTS

Tests for the cause of the acute nephritic

syndrome may include:
Culture of the throat or skin
Blood culture
ANA titer (lupus)
Serum complement (C3 and C4)
ANCA (antineutrophil cytoplasmic antibody for
vasculitis)
Anti-glomerular basement membrane
antibody
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Chronic Glomerulonephritis
Chronic glomerulonephritis describes a patient with
evidence of chronic renal failure who shows
characteristics of glomerular disease.
The urinalysis demonstrates a few RBCs and WBCs but
is mostly nonspecific.
A variable amount of proteinuria is present.
Kidney size is typically small, reflecting the presence
of advanced fibrosis and glomerulosclerosis. Patients
with this syndrome presumably have a glomerular
disease that has progressed to end stage, leaving the
kidneys irreversibly damaged.

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Rapidly Progressive
Glomerulonephritis

Rapidly progressive glomerulonephritis should be

considered in patients who present with a nephritic
clinical picture and who have a nephritic urinary
sediment.
This condition is distinguished from acute
glomerulonephritis by the rapid loss of renal function,
which is defined as a rise in the serum creatinine
concentration of more than 2 mg/dl over a 3-month
period.
This syndrome has a much less consistent temporal
relationship with infection, and there is little tendency
for spontaneous recovery.
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Rapidly Progressive
Glomerulonephritis

This syndrome needs to be recognized early so that

renal biopsy can be done and therapy instituted
immediately, if indicated.

With patients in whom renal biopsy shows a

crescentic glomerulonephritis, immunofluorescent
studies provide a useful classification of the diseases
that most commonly give rise to this clinical
syndrome.

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COMPLICATIONS

Acute kidney failure

Chronic kidney failure
End-stage kidney disease
High blood pressure
Congestive heart failure
Pulmonary edema
Chronic glomerulonephritis
Nephrotic syndrome

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PROGNOSIS

Acute nephritic syndrome resolves completely in

about 80 to 90% of children and about 60% of adults.
The prognosis for people with rapidly progressive
glomerulonephritis depends on the severity of
glomerular scarring and whether the underlying
cause, such as infection, can be cured.
In about half of the people who are treated early
(within weeks to a few months), kidney function is
preserved and dialysis is not needed.

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PROGNOSIS
However, because the early symptoms can be subtle
and vague, many people who have rapidly progressive
glomerulonephritis are not aware of the underlying
disease and do not seek medical care until kidney
failure develops.
People with advanced kidney failure die within a few
weeks unless they undergo 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 nephritic syndrome, other types
of kidney disorders develop, such as asymptomatic
proteinuria and hematuria syndrome or nephrotic
syndrome.

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TREATMENT

No specific treatment is available in most cases of

acute nephritic syndrome.
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 nephritic syndrome, antibiotics are usually
ineffective because the nephritis begins 1 to 6 weeks
(average, 2 weeks) after the infection. However, if a
bacterial infection is still present when acute nephritic
syndrome is discovered, antibiotic therapy is started.
Antimalarial drugs may be beneficial if the cause of the
syndrome is malaria.
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TREATMENT

For rapidly progressive glomerulonephritis, drugs to

suppress the immune system are started promptly.
High doses of corticosteroids
Cyclophosphamide
plasmapheresis is sometimes used to remove
antibodies from the blood
dialysis
Kidney transplantation is sometimes considered for
people who have chronic kidney failure, but rapidly
progressive glomerulonephritis may recur in the
transplanted kidney.
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TREATMENT

For rapidly progressive glomerulonephritis, drugs to

suppress the immune system are started promptly.
High doses of corticosteroids
Cyclophosphamide
plasmapheresis is sometimes used to remove
antibodies from the blood
dialysis
Kidney transplantation is sometimes considered for
people who have chronic kidney failure, but rapidly
progressive glomerulonephritis may recur in the
transplanted kidney.
Angiotensin-converting enzyme (ACE) inhibitors often
slow progression of chronic nephritic syndrome.
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