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GLOMERULAR SYNDROMES

LIGIA PETRESCU


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BACKGROUND

This class of kidney disease centers around theglomerulus.This is where the main filtration of thenephron occurs and is located within theBowman's capsule.

It is comprised of a mass of tiny tubes throughwhich the blood passes.Its semipermeable structure allows water andsoluble wastes to pass through and beexcreted out of the Bowman's capsule as urine.

The filtered blood passes out of theglomerulus into the efferent arteriole to bereturned through the medullary plexus to theintralobular vein.


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CLASIFICATION

ACUTE NEPHRITIC SYNDROME

RAPIDLY PROGRESSIVE

GLOMERULONEPHRITIS NEPHROTIC SYNDROME

ASYMPTOMATIC HEMATURIA ORPROTEINURIA

CHRONIC GLOMERULONEPHRITIS


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ACUTE NEPHRITIC SYNDROME

DEFINITION AND CLASIFICATION

Acute nephritic syndrome is a group of disorders that causeinflammation of the internal kidney structures (specifically,the glomeruli), often caused by an immune responsetriggered 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, redcell casts, proteinuria, renal failure.

Nephritic syndrome can develop suddenly or over a shorttime period (acute nephritic syndrome) or develop andprogress slowly (chronic nephritic syndrome).

In 1% of children and 10% of adults, the acute nephriticsyndrome evolves into rapidly progressiveglomerulonephritis, in which most of the glomeruli aredestroyed, resulting in kidney failure


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ETIOLOGY

The cause of chronic nephritic syndromecannot be identified in many people.

Often, chronic nephritic syndrome seems toresult from one of the same conditions thatcauses acute nephritic syndrome.


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PRIMARY GLOMERULOPATHIES

ACUTE DIFFUSE PROLIFERATIVEGLOMERULONEPHRITIS

RAPIDLY PROGRESSIVE (CRESCENTIC)GLOMERULONEPHRITIS

MEMBRANOUS GLOMERULOPATHY

MINIMAL CHANGE DISEASE

FOCAL SEGMENTAL GLOMERULOSCLEROSIS

MEMBRANOPROLIFERATIVEGLOMERULONEPHRITIS

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


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HEREDITARY DISEASES

ALPORT SYNDROME

THIN MEMBRANE DISEASE

FABRY DISEASE


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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 COMPLEXDEPOSITION

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 acutenephritis below, they share many symptoms incommon.

About half of the people with acute nephriticsyndrome have no symptoms.

If symptoms do occur, the first to appear are fluidretention and tissue swelling (edema), low urinevolume, 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, visualdisturbances, and more serious disturbances of brainfunction.

In older people, nonspecific symptoms, such asnausea and a general feeling of illness (malaise), aremore common.

When rapidly progressive glomerulonephritisdevelops, weakness, fatigue, and fever are the mostobvious early symptoms. Loss of appetite, nausea,vomiting, abdominal pain, and joint pain are alsocommon.

About 50% of people had a flu-like illness in themonth before kidney failure started to develop.These people have edema and usually produce verylittle urine. High blood pressure is uncommon andrarely severe when it does occur.


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SYMPTOMS

Because chronic nephritic syndrome usually causesonly very mild or subtle symptoms for years, it goesundetected in most people.

Fluid retention (edema) may occur. High blood pressure is common.

The disease may progress to kidney failure, which cancause 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 incirculation than the heart can effectively pump),

including abnormal heart and lung sounds. Thejugular (neck) veins may be distended from increasedpressure.

Generalized swelling is often present. When

examining the abdomen, may be signs of fluidoverload and an enlarged liver.
http://www.nlm.nih.gov/medlineplus/ency/article/003275.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003275.htm


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SIGNS AND TESTS

There may be signs of acute kidney failureinaddition to the above symptoms.

Urine appearance and colorare abnormal (coca-cola).

Urinalysis nephritic urinary sediment revealsvariable number of red blood cells (RBCs), WBCs,and RBC casts (pathognomonic of activeglomerulonephritis)

Protein in the urinetest is positive; Proteinuria is

usually modest, ranging from 2 to 6 g in a 24-hourcollection
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SIGNS AND TESTS

Azotemia (nitrogen-containing waste products in theblood) may be evidenced by: elevated BUN elevated creatinine

potassium testmay be elevated.

The creatinine clearancemay be decreased. kidney biopsy reveals glomerulonephritis

(inflammation of the glomeruli), confirm thediagnosis, help determine the cause, and determinethe 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 informationabout the cause is small.
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SIGNS AND TESTS

Tests for the cause of the acute nephriticsyndrome may include:

Culture of the throator skin

Blood culture

ANAtiter (lupus) Serum complement(C3and C4)

ANCA (antineutrophil cytoplasmic antibodyforvasculitis)

Anti-glomerular basement membraneantibody
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Chronic Glomerulonephritis

Chronic glomerulonephritis describes a patient withevidence of chronic renal failure who showscharacteristics 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. Patientswith this syndrome presumably have a glomerulardisease that has progressed to end stage, leaving thekidneys irreversibly damaged.

idl i


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Rapidly ProgressiveGlomerulonephritis

Rapidly progressive glomerulonephritis should beconsidered in patients who present with a nephriticclinical picture and who have a nephritic urinarysediment.

This condition is distinguished from acuteglomerulonephritis by the rapid loss of renal function,which is defined as a rise in the serum creatinineconcentration of more than 2 mg/dl over a 3-monthperiod.

This syndrome has a much less consistent temporal

relationship with infection, and there is little tendencyfor spontaneous recovery.


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Rapidly ProgressiveGlomerulonephritis

This syndrome needs to be recognized early so thatrenal biopsy can be done and therapy institutedimmediately, if indicated.

With patients in whom renal biopsy shows acrescentic glomerulonephritis, immunofluorescentstudies provide a useful classification of the diseasesthat most commonly give rise to this clinicalsyndrome.


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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 inabout 80 to 90% of children and about 60% of adults.

The prognosis for people with rapidly progressiveglomerulonephritis depends on the severity of

glomerular scarring and whether the underlyingcause, such as infection, can be cured. In about half of the people who are treated early

(within weeks to a few months), kidney function ispreserved 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 progressiveglomerulonephritis are not aware of the underlyingdisease and do not seek medical care until kidneyfailure develops.

People with advanced kidney failure die within a fewweeks unless they undergo dialysis.

The prognosis also depends on the cause, the person'sage, and any other diseases the person might have.When the cause is unknown or the person is older, theprognosis is worse.

In some children and adults who do not recover

completely from acute nephritic syndrome, other typesof kidney disorders develop, such as asymptomaticproteinuria and hematuria syndrome or nephroticsyndrome.

TREATMENT


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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 usuallyineffective because the nephritis begins 1 to 6 weeks(average, 2 weeks) after the infection. However, if abacterial infection is still present when acute nephritic

syndrome is discovered, antibiotic therapy is started.Antimalarial drugs may be beneficial if the cause of thesyndrome is malaria.


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TREATMENT

For rapidly progressive glomerulonephritis, drugs tosuppress the immune system are started promptly.

High doses of corticosteroids

Cyclophosphamide

plasmapheresis is sometimes used to removeantibodies from the blood

dialysis

Kidney transplantation is sometimes considered forpeople who have chronic kidney failure, but rapidly

progressive glomerulonephritis may recur in thetransplanted kidney.


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TREATMENT

For rapidly progressive glomerulonephritis, drugs tosuppress 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 rapidlyprogressive glomerulonephritis may recur in the

transplanted kidney. Angiotensin-converting enzyme (ACE) inhibitors often

slow progression of chronic nephritic syndrome.

sdr glomerular

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