its about the autoimmune disease.. the IgA nephropathy..

2. AUTOIMMUNE DISEASE:
Arises from abnormal immune response
of the body
May be restricted to certain organs eg
AUTOIMMUNE THYROIDITIDS
May involve a particular tissue which
may affect the basement membrane in
both lungs and kidney. eg good Pasteur’s
disease

3. CRITERIA:-
For a disease to be recognized as an
autoimmune disease it must answer
WITEBSKY’S POSTULATES (formulated
by ERNST WITEBSKY & COLLEAGUES IN
1957 AND MODIFIED IN 1994).

4. POSTULATES:-
Indirect evidence based on reproduction of autoimmune
disease in experimental animals.
Direct evidence from transfer of pathogenic antibodies
or pathogenic T-cell
Circumstanicial evidences from clinical clues.
Genetic architechture clustering with other autoimmune
disease

6. DEFINITION:-
Primary IgA Nephropathy is
characterized by the deposition of
IgA antibody in the glomerulus, i.e
it is a kidney disease that occurs
when IgA (immunoglobulin A)
lodges in your kidney. This results
in local inflamation that, over
time, may hampers kidneys ability
to filter waste, excess water and
electrolytes from blood.

7. ALSO CALLED AS:-
Berger’s disease
IgA nephritis
IgAN
Berger’s syndrome
Synpharyngitic
glomerulonephritis

8. HISTORY:-
Immunoglobulin A (IgA)
nephropathy was first described
by the pathologist JEAN BERGER
and thus is called BERGER’S
DISEASE.
JEAN BERGER published his
description of glomerulo-nephritis
with mesangial IgA deposition in
winter 1968.

9. EPIDEMIOLOGY
Occur in any age but more common in children and
young adult
Male>female (2 folds)
Genetic predisposition: IGAN1, on 6q22-q23
Susceptibility to IgA nephropathy
has been associated with single
nucleotide polymorphisms (SNPs) in
the E-selectin and L-selectin genes as
well as in polymeric
immunoglobulin receptor gene
(PIGR) .

11. IgA NEPHROPATHY
•It is typically an isolated renal disease
•IgA deposits are present in systemic
disorder of children, HENOCH-
SCHONLEIN purpura which has
many overlapping features with IgA
nephropathy
•Secondary IgA nephropathy occurs in
patients with liver and intestinal
diseases.

12. Signs and
Symptoms of
IgA
Nephropathy

13. of IgA
Nephropathy

14. Early stages of disease
IgA nephropathy
usually doesn't cause
symptoms in the early
stages. The disease can
go unnoticed for
decades and is
sometimes first
suspected when
routine tests reveal
protein and red blood
cells in your urine that
can't be seen without a
microscope
(microscopic
hematuria).

15. Signs and symptoms of IgA
nephropathy when kidney
function is impaired include:
Cola- or tea-colored urine
(caused by red blood cells in
the urine)
Repeated episodes of cola- or
tea-colored urine, sometimes
even visible blood in your
urine, usually during or after
an upper respiratory or other
type of infection
Pain in the side(s) of your
back below your ribs (flank)
Foam in the toilet water from
protein in your urine
Swelling (edema) in your hands
and feet
High blood pressure

16. Pain in the side(s) of your back
below your ribs (flank)
Foam in the toilet water from
protein in your urine
Swelling (edema) in your hands and
feet
High blood pressure

17. When to see a doctor :
Make an appointment with your doctor if you
notice blood in your urine. Urinary bleeding
may be caused by strenuous exercise, some
foods, medications or a urinary tract
infection. But prolonged or repeated bleeding
may indicate a serious medical problem and
should always be evaluated. Also see your
doctor if your urine is foamy or if you develop
sudden swelling in your hands and feet.

18. DIAGNOSIS OF IGA NEPHROPATHY
IgA nephropathy is often detected after we notice blood in our urine
or when a routine test shows we have protein(proteinuria) or
blood(microhematuria) in our urine. These could be signs of several
types of kidney disease. To identify problem, these tests may be
performed:
URINE TEST:
Blood or protein in the urine may be the first sign of IgA
nephropathy. This may be discovered as part of a routine checkup.
If doctor suspects that you have problems with your kidneys, you
may need to collect your urine for a 24-hour period for additional
kidney function tests.

19. BLOOD TEST:
If you have kidney disease, such as IgA
nephropathy, a blood test may show
increased blood levels of the waste
product creatinine. Based on a 24-hour
urinalysis, this test ascertains how well
kidneys are performing their vital task
of clearing creatinine, the end product
of muscle metabolism, from the blood.
The results show the
kidneys’ glomerular

20. This test is not diagnostic of a particular
disease but, rather, of the severity of the
inflammation and, possibly, the extend of
damage to the kidneys. Your kidneys
ordinarily filter about five ounces of blood
each minute, but in IgA Nephropathy, the
filtration rate may decline because
infiltration of the glomerular capillaries by
inflammatory cells reduces the amount of
surface area available for filtering work.
Blood and urine testing and the absence of
certain hallmarks of other kidney diseases,
such as azotemia [excessive nitrogen in the
blood] and pronounced edema [swelling of
tissues from retained fluid], may suggest
IgA Nephropathy, but only a biopsy can
confirm it.

21. KIDNEY BIOPSY:
The only way for your doctor to
confirm a diagnosis of IgA
nephropathy is with a kidney
biopsy. This procedure involves
using a special biopsy needle to
extract small pieces of kidney
tissue for microscopic
examination to determine if
there are IgA deposits in the
glomeruli.

22. RENAL IMAGING:
It is a diagnostic procedure that uses
gamma rays to examine the anatomy
and functioning of kidneys. During this
procedure, radioisotope releases gamma
rays, which a gamma camera or scanner
can detect from outside the body. The
gamma camera scans the kidney area.
The camera also works together with
the computer to create images. These
images detail the structure and
functioning of kidneys. Images from a
renal scan can show structural and
functional abnormalities, helping
doctors to diagnose a kidney problem.

24. The course of IgA nephropathy varies from
person to person. Some people have the
disease for years with few problems. In fact,
many cases may go undiagnosed. Other
people develop one or more of the following
complications:
•High blood pressure. Damage to your
kidneys from IgA deposits can raise your
blood pressure, and high blood pressure can
cause further damage to your kidneys.
•High cholesterol. High levels of cholesterol
may increase your risk of a heart attack.

25. •Acute kidney failure:
If your kidneys lose their filtering ability due to IgA deposits, waste products build
up quickly in your blood.
•Chronic kidney failure:
IgA nephropathy can cause your kidneys to gradually stop functioning. In such
cases, permanent dialysis or a kidney transplant is needed to sustain life.
•Nephritic syndrome:
This is a group of problems that can be caused by damage to the glomeruli,
including high urine protein levels, low blood protein levels, high cholesterol and
lipids, and swelling of your eyelids, feet and abdomen.

26. Researchers don't know exactly what causes IgA deposits in the kidneys,
but these conditions or factors may be associated with the development
of IgA nephropathy:
•Genes, because IgA nephropathy is more common in some families
and in certain ethnic groups
•Liver diseases, including cirrhosis, a condition in which scar tissue
replaces normal tissue within the liver, and chronic hepatitis B and C
infection.
•Celiac disease, a digestive condition triggered by eating gluten, a
protein found in most grains
•Dermatitis herpetiformis, an itchy, blistering skin disease that stems
from gluten intolerance
• Infections, including HIV infection and some bacterial infections

27. THERAPY:-

28. IgA nephropathy is a common cause of
glomerulonephritis. Although it is a benign
disease in most patients, chronic kidney
disease and end-stage renal disease (ESRD)
occur in about 20-40% of patients within 20
years of presentation. Currently, no cure exists
for IgA nephropathy, but therapies that can
delay the onset of need for dialysis and
transplantation are available. Current
recommendations include the following:

29. Monitor patients with isolated hematuria without proteinuria or hypertension
with urinalysis, renal function testing, and blood pressure measurement.
Treat hypertension early and aggressively. A reasonable goal is to aim for a
blood pressure of 125/75–130/80 mm Hg.
Angiotensin-converting enzyme inhibitors (ACEIs) are the preferred agents
for lowering blood pressure. They are beneficial in decreasing proteinuria and
should be strongly considered even in normotensive patients with
proteinuria.The decrease in proteinuria with ACEIs may be an effect of
decreasing the intraglomerular pressure and of changing the glomerular size
selectivity.
Reports have demonstrated that ACEIs are more effective than other
antihypertensive drugs in slowing the progression of proteinuric renal disease.

30. The goal for proteinuria control is 500 mg/day or less, so ACEIs should be
started in patients with 24-hour urine protein of 500 mg or more.[7]
In a randomized, controlled trial that followed patients for a mean of
approximately 6 years, the group that received ACEIs had an improved renal
survival rate compared with the group receiving other antihypertensive agents.
Angiotensin II receptor blockers (ARBs) should be used for patients who cannot
tolerate ACEIs. ACEIs and ARBs may have an additive effect in decreasing
proteinuria. Whether high-dose ACEIs better preserve renal function than
combination therapy with ACEIs and ARBs is unknown.
The combination of an ACEI and the ARB losartan has shown an additive
urinary protein–lowering effect compared with doubling the dose of
monotherapy. However, patients on combination therapy should be monitored
closely for the development of hyperkalemia, and combination therapy should be
avoided in patients with advanced kidney disease.

31. Administer prednisone for 4-6 months to patients who have IgA
nephropathy with preserved renal function, nephrotic syndrome, and
minimal-change findings on light microscopy.
Early treatment with prednisone in patients with proliferative IgA
nephropathy has been shown to be effective in reducing proteinuria
and improving histologic findings, such as proliferation and
cellular crescents. Additionally, corticosteroids given for 6 months
have been seen to be beneficial against deterioration in renal function
in patients with moderate proteinuria (1.5-3.5 g/d).
Results of a prospective, open-label, multicenter, centrally
randomized, controlled trial in 97 patients suggested that the
combination of the ACEI ramipril and prednisone was more effective
than ramipril alone in discouraging progression of renal disease
associated with IgA nephropathy.

32. A randomized, controlled, long-term study on the effectiveness of steroids in
IgA nephropathy showed improved 10-year renal survival in the steroid-treated
group compared with the control group. Patients had a proteinuria of 1.9 g/d on
average in the treatment group and 1.7 g/d in the control group. Steroids were
given for 6 months.
Mycophenolate mofetil has been used in patients with IgA nephropathy
associated with proteinuria, even though some reports have shown some benefit
and others have not. The studies are of small size, and longer-term studies are
required for more information. At this time, the evidence for the use of
mycophenolate in IgA nephropathy is inconclusive.
Patients with crescentic rapidly progressive glomerulonephritis (RPGN) can be
treated similarly to patients with idiopathic RPGN by using intravenous pulse
prednisone followed by oral prednisone and cyclophosphamide.

33. Fish oil (omega-3 fatty acids) at a dose of 12 g/d has been used with
controversial and conflicting results, but it is frequently used in patients with
declining renal function. Deficiencies of essential fatty acids have been
detected in IgA nephropathy, and fish oil is rich in long-chain omega-3
polyunsaturated fatty acids. These produce altered and less biologically
effective prostaglandins and leukotrienes, as well as reduced platelet
aggregation.
A study by Liu examined the effect of calcitriol on urinary protein excretion
among patients with IgA nephropathy. The study found that adding calcitriol
to a renin-angiotensin system inhibitor resulted in a safe decrease in
proteinuria.