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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.
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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.