| What
is IgA Nephropathy?
Think of your kidneys as small but
powerful filtration plants whose job is to keep your blood
clean and the body’s chemical balance maintained. Each
day the kidneys process about 200 quarts of fluid through
their two million tiny treatment plans, the nephrons.
Within the nephron is the glomerulus, a
tangle of fine capillaries that filter the blood before
passing it on to the tubules, where the
kidneys continually adjust the filtrate to your body’s
needs, adding back chemicals removed during filtration or
drawing off more water. What’s needed by the body is
returned to the bloodstream; what’s not needed is
excreted as urine.
In IgA Nephropathy [IgAN],
unknown agents cause the glomeruli to become
— and to stay — inflamed. IgAN is the world’s most
common glomerulonephritis [inflammation of
the glomeruli], but its pathogenesis [how
the disease develops] is not known. IgAN is considered to
be an immune-complex mediated disorder (or
immunologically mediated disorder), which means that
immune complexes may not be the direct cause of the
disease but they help bring about the end result, which is
widespread inflammation of the kidneys.
Immune complexes form when the body is
exposed to an antigen, such as a virus,
bacteria, toxin, or even allergen. In response, your body
sends out antibodies, which are
immunoglobulins produced by a certain class of white blood
cells known as B-cells. There are five main classes of
these immunoglobulins, with IgA [immunoglobulin
A] being the main one directed against bacterial and viral
antigens.
Antibodies lock onto the surfaces of
viruses and other foreign cells, producing
antigen-antibody aggregates known as immune
complexes that your body targets and attacks.
Normally, these immune complexes circulate through the
bloodstream until being removed by the liver and other
organs. In IgAN, however, they become trapped in the
glomeruli.
Once trapped, immune complexes become
like magnets for the rest of the body’s immune response.
Macrophages and other cells race to the area to release
enzymes and generate toxic oxygen radicals that kill
bacteria and viruses but also injure local tissues.
Trapped immune complexes can also stimulate the
overproduction of extracellular matrix, a
substance which surrounds and supports tissue cells.
Inflammatory chemicals released during the immune response
increase the permeability of the glomerular capillary
walls, causing blood being filtered in the kidneys to leak
protein and fibrinogen. Fibrinogen, crucial
to the process by which blood clots, develops into "crescents,"
which form scar tissue that obstructs circulation through
the glomerulus. Eventually, deprived of blood, the tubules
and glomerulus die, thus destroying the nephron and
forcing other nephrons to take up its work.
What happens in IgAN is essentially a
vicious cycle: inflammatory chemicals increase the
permeability of the glomerular capillary wall, and that
increased porosity enhances the absorption of other
chemicals that stimulate cellular growth and, ultimately,
structural damage. These changes take time because our
kidneys have a lot of excess capacity built into them. The
damage is, however, slowly progressive and, in the case of
sclerosis [scarring], irreversible.
We do not know what antigen is
responsible for triggering IgAN, nor do we know whether
IgAN results from defects in the body’s creation or
elimination of immune complexes. There is evidence that
immunoglobulin A is overproduced in the bone marrow of
IgAN patients; some patients also show decreased capacity
to remove immune complexes from their systems.
Because so many IgAN patients have a
cold or gastrointestinal illness shortly before the onset
of symptoms in the kidneys, it is likely that IgAN starts
with an immune response to infectious agents. Perhaps
protracted exposure to antigens — as in a recurrent
viral illness, or the multiple illnesses experienced by
some IgAN patients — sends the immune system into
overdrive. The blood of IgAN patients often shows high
concentrations of IgA antibodies to a type of protein
found in many bacteria, as well as antibodies to many
viruses. Certain viruses, once established in the body as
a low-level chronic infection, could serve as a continuing
source of antigen for the formation of additional IgA
immune complexes — and for the perpetuation of injury to
the kidneys. It is quite common for IgAN to present after
an individual has had an illness such as strep throat, ear
infection, etc., that was treated with antibiotics.
Antibiotics are effective against bacteria but not against
viruses and can even suppress the immune system as far as
viral infections are concerned, perhaps allowing a viral
infection to become chronic.
Viruses can also over-sensitize the
immune system, inducing autoimmune reactions in which the
body becomes allergic to itself. Few IgAN patients,
however, show a positive anti-nuclear antibody test
[ANA], which is an important tool in diagnosing such
autoimmune diseases as systemic lupus erythematosus.
It’s clear that IgAN is not simply a
kidney disease but rather an immunological disorder that
affects the kidneys, as diabetes is an immunological
disorder that affects the pancreas. It may also be a
disease, like diabetes, that affects the micro blood
vessels. If true, this would explain some common
extra-renal symptoms, such as headaches, temporary vision
problems, and difficulties concentrating.
^^^top^^^
How
is IgA Nephropathy diagnosed?
In countries like Japan, where routine
urinary screening of school children is required by law,
the disease is often picked up at a very early stage by
the presence in the urine of protein [proteinuria]
or of microscopic amounts of blood [microhematuria].
In the United States, it is most commonly discovered after
a child or adult passes a strange urine discolored by
blood [gross or macrohematuria] so it looks
like murky Coca-Cola or tea. In the West, IgAN tends to be
discovered more readily among girls, perhaps because they
are at greater risk for urinary tract infections and more
likely to undergo urinalysis than boys.
Blood in the urine can be caused by many
things. Because so few doctors are familiar with IgAN, a
patient may be put through extensive urological tests to
determine if there is a urinary tract infection or
congenital abnormality. But if the red blood cells in the
urine are examined under a microscope, they appear dysmorphic
[strangely shaped]: instead of being full and round, they
look as if they’d been nibbled around the edges. This is
a tip-off that glomerulonephritis is involved, but it does
not tell the doctor which glomerulonephritis it might be.
A physician may first believe that this
is post-streptococcal glomerulonephritis [PSGN],
a disease that resembles IgAN in some ways (but which,
unlike IgAN, tends to resolve itself spontaneously). To
assist the diagnosis, the doctor performs a variety of
tests, including a complete blood count,
blood-urea-nitrogen [BUN], serum creatinine,
and ASO titers to rule out the possibility of
post-streptococcal glomerulonephritis.
Your doctor may also order a creatinine
clearance test. Based on a 24-hour urinalysis,
this test ascertains how well they kidneys are performing
their vital task of clearing creatinine, the end product
of muscle metabolism, from the blood. The results show the
kidneys’ glomerular filtration rate. 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 IgAN, 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
IgAN, but only a biopsy can confirm it.
^^^top^^^
Is
a biopsy really necessary?
Probably not. If a case seems mild, a
doctor may recommend following it through periodic office
visits. If the clinical symptoms are severe, however, a
doctor is more likely to recommend a biopsy.
Only by examining tissue samples under
light and electron microscopy and immunofluorescence can
doctors confirm a presumptive diagnosis of IgAN. If a
patient has IgAN, deposits of IgA [immunoglobulin
A] show up in a definite pattern within sections of the
glomeruli, usually the mesangium. Perhaps
more important, only by examining samples of kidney tissue
can doctors tell how much damage has occurred. The nature
and extent of damage are important in determining the
likely prognosis [outcome of the disease].
In theory this should influence the
doctor’s choice of treatment, but in practice it rarely
does. Ask your doctor whether having a definitive
diagnosis from a biopsy is likely to make any difference
in his approach to treating IgAN before agreeing to a
biopsy.
Biopsy specimens of IgAN patients show a
wide variety of lesions [changes to the
structure of tissues], the most common being mesangial
proliferation, in which the mesangium supporting
the glomerular capillaries becomes widened by excessive
growth of matrix. The pattern can be focal [affecting
all of a few glomeruli in an area], or segmental
[affecting only certain parts of the glomeruli], or diffuse
[affecting all of every glomerulus]. Damage can affect
other parts of the nephron, such as the tubules or Bowman’s
capsule, which houses the glomerulus. Under
immunofluorescence, technicians see IgA deposited in
granular patterns. Other immunoglobulins, complement, and
fibrin may also be present, as well as evidence of sclerosing
[scarring].
The more severe the clinical findings
— especially if proteinuria is very high — the more
severe the biopsy results are likely to be. Because there
is no uniform system for grading biopsy specimens (and
because the terms used are confusing even to researchers),
it’s a good idea to discuss the biopsy results
thoroughly with your doctor and get an explanation of the
grading system used by the laboratory in evaluating the
lesions. Needless to say, you should also obtain a copy of
the biopsy report for your medical records.
The renal biopsy almost always used in
the U.S. is the percutaneous, in which a tiny core of
tissue is removed through a hollow biopsy needle inserted
into the kidney through the back. To guide the needle into
place, the doctor uses such techniques as intravenous
pyelography, radionuclide scans, fluoroscopy, and
especially ultrasound (which involves no radiation
exposure). Done under sedation and local anesthetic,
percutaneous biopsy involves an overnight stay at the
hospital.
Although a renal biopsy is considered a
minor surgical procedure, it can create complications.
Risks include developing an infection or having bleeding
around or within the kidney, with subsequent development
of a hematoma [a swelling filled with
blood]. It is possible that pressure from a large hematoma
might hasten deterioration of the kidneys. A more likely
problem, however, is that the nephrologist might not
obtain enough tissue from the glomeruli for adequate
analysis.
Because the risk of complications is
higher in pediatric biopsies (about one-third of these
experience complications), it is important to weigh
carefully whether a biopsy is REALLY necessary in the case
of a child, especially a young one. Some hospitals permit
parents to attend biopsies, but this is not something to
be undertaken lightly, for it can be upsetting to watch
your child reacting to the needle, even though under
sedation. If at all possible, parents should stay with a
child after a biopsy, because there is likely to be some
post-op pain and discomfort.
For anyone undergoing a biopsy, it is
important to drink plenty of water or juices in the hours
following the procedure.
^^^top^^^
How
does one get IgA Nephropathy?
At present, we don’t know how the
disease is contracted, or how it develops, or how to
protect ourselves against it. We do know that it is not
contagious.
Its development may be linked to
possible genetic defects in immune response or
immune-complex clearance. By themselves, these do not
cause disease. Some type of antigen must be present in the
body, perhaps bacterial (streptococcus), or viral
(influenzas of certain type), or endogenous (produced by
the body itself). Genetic programming, however, may make
some individuals more susceptible to developing IgAN upon
exposure to certain antigens.
People who have IgAN rarely have a
history of kidney disease in their family. Often, however,
there is a personal or family history of other
immunologically-mediated disorders, such as psoriasis,
asthma, or celiac-sprue, especially if these have been
treated with such immunosuppressive medications as
corticosteroids. If the family’s medical history
includes such disorders, or if other family members have
experienced "brown urine" or excessive fatigue,
the Network recommends screening the blood pressure and
urine of all family members.
^^^top^^^
How
many people have IgA Nephropathy?
Kidney diseases of all types affect over
13 million Americans, killing about 78,000 each year. In
1989, Medicare’s End-Stage Renal Diseases Program
accepted nearly 42,000 new patients, primarily for
dialysis. Of these, 13.1% had glomerulonephritis, which
ranks behind only diabetes and hypertension as a
contributor to renal failure.
Although glomerulonephritis is a major
cause of kidney failure — and IgAN is by far the most
common glomerulonephritis — we have no firm idea how
many people are affected by IgAN. One reason for this is
that definitive diagnosis of IgAN is possible only by
biopsy, but biopsies are not conducted routinely for mild
urinary abnormalities. Another reason is that there is no
separate ICD (International Classification for Diseases)
code for IgAN. Hospitals use these codes to classify their
diagnoses, and government agencies use such data to track
the incidence [number of new cases each
year] of various diseases.
In 1991, the Centers for Disease Control
(CDC) estimated that the prevalence [total
number of cases] of IgAN was small in the United States,
perhaps on the order of 5,000 people. The next year the
CDC raised that estimate to 150,000. This does not mean
that we have a sudden epidemic of IgAN in America; it does
mean that public health officials are becoming more aware
of this disease and concerned about its potential impact.
According to Medicare data for
1986-1989, glomerulonephritis causes approximately 5,500
cases of kidney failure each year. The National Institutes
of Health’s Division of Kidney, Urologic &
Hematologic Diseases believes that the "overwhelming
majority" of those diagnosed with glomerulonephritis
have IgA Nephropathy. Others estimate that IgAN is
responsible for 10-20% of all end-stage renal disease.
These estimates suggest that IgAN may be sending 2,800 to
4,200 Americans into renal failure each year.
The prevalence of IgAN is thought to be
lower in North America than in Asia, Australia, and parts
of Europe, but it is not clear whether this difference is
real or merely reflects an understandable reluctance by
doctors in the U.S. to submit patients to biopsy for signs
such as asymptomatic microhematuria [traces
of blood in the urine with no other symptoms].
In the United States, up to 5% of renal
biopsies examined by immunofluorescence are diagnosed as
IgAN. Yet in Japan, where schoolchildren have a mandatory
urinalysis each year, and in Singapore, where all young
men are screened for military service, IgAN accounts for
up to 50% of glomerulonephritis cases. These are among the
highest rates in the world, but whether they reflect a
genetic predisposition to the disease, or environmental
factors, or simply better screening and earlier diagnosis,
no one knows.
Similarly, Finland, which also screens
its young men for compulsory military service, detects an
average of 22 cases of IgAN per 100,000 young male
population per year. Presumably, this does not include
cases found among females or older men, although IgAN
typically strikes more men than women (the ratios vary by
country) and strikes most often in the very early teens or
twenties. Other European nations, like Scotland, are
finding they have more IgAN than previously supposed, and
this is probably happening in the United States as well.
IgAN is seldom seen in blacks, although
its presumed rarity in Africa may be owing to
underdiagnosis. In areas like Singapore, there is no
significant difference in incidence among the Malay,
Chinese, and Indian populations. American Indians,
especially the Zuni, have an unexpectedly high incidence
of IgAN, but, again, whether due to genetic or
environmental factors, no one knows. Even among Native
Americans, some doctors believe the incidence is grossly
underestimated because so many Indians do not receive
proper medical care or are never biopsied.
In the U.S., IgAN is considered an
"orphan disease," one affecting fewer than
200,000 people. Underestimated though its prevalence may
be, IgAN will no doubt always be viewed as a rare disease.
That makes it more difficult to attract governmental and
private funding for research, to conduct trials of various
treatments, and to link patients for mutual support.
^^^top^^^
What
are the major symptoms of IgA Nephropathy?
The most frequently seen symptoms are proteinuria
and hematuria. Hematuria is the presence of
blood in the urine, either as scattered cells discernible
only by microscopic analysis or chemical tests (microhematuria),
or visible to the eye as a darkening of the urine (macro-
or gross hematuria).
Rarely in episodes of macrohematuria
does the urine look red or "bloody";
usually it is tea-colored or smoky. It may also have a
strong smell. Red blood cells predominate; the presence of
white cell casts usually indicates inflammation of the
glomeruli.
Seen in a toilet bowl, diluted by water,
this dark urine may give the impression that you’re
dehydrated. But urine from dehydration is dark yellow or
orange in color, whereas urine from macrohematuria has a
dark green or black tinge. If there is any doubt, void
into a clear plastic cup: the differences in appearance
between macrohematuria and dehydrated urine are more
striking there than in the toilet bowl.
We do not know what causes hematuria or
what its presence in the urine means. Although gross
hematuria may look scary, patients who have it generally
are considered to have a better prognosis than those with
persistent microhematuria. Those presenting with gross
hematuria will probably have recurrences at intervals
ranging from months or years, with the intervals growing
longer over time. Even those who did not initially
experience gross hematuria are likely to have an episode
at least once. Episodes are often closely associated with
a respiratory infection, less frequently with a
gastrointestinal infection, strenuous exercise, or
vaccination.
During episodes of gross hematuria, it
is important to drink lots of pure water to prevent the
formation of tiny blood clots that can behave much like
kidney stones and potentially cause pain. Expect to feel
generally rotten during the course of an episode. An
episode’s length varies from patient to patient, but
most last a few days.
A very, very few patients experience
constant gross hematuria of pure blood. Their urine looks
red rather than smoky. Such patients should NOT use fish
oil or any medication that acts as an anticoagulant
(including aspirin), as it can worsen their bleeding.
Proteinuria
is the leakage of protein into the urine. Ordinarily, the
glomerular capillary walls behave like a fine sieve, but
inflammation in effect loosens the sieve’s mesh,
allowing larger molecules to escape. The normal glomerular
capillary wall is charge-selective as well, for portions
of its basement membrane carry a negative
electrical charge. This becomes neutralized in IgAN,
possibly by positively-charged enzymes released by
inflammatory cells. When this occurs, the capillary walls
no longer repel negatively-charged protein molecules,
allowing some to escape into the urine.
A person can be considered healthy and
still excrete a small amount of protein in the urine (up
to 150 mg/day [milligrams per day], or 0.15 g/day
[grams per day]). Losing more than that is abnormal.
Proteinuria is usually defined as heavy (over 3,000
mg/day), moderate (1,000-2,900 mg/day), and mild
(under 1,000 mg/day). Many patients with IgAN, especially
children, have only mild to moderate proteinuria.
Proteinuria can also be
"selective" (containing only proteins of certain
molecular weights) or "nonselective" (containing
mixed-weight proteins). Generally speaking, those patients
with selective proteinuria, especially if almost
exclusively albumin, have a better prognosis than those
whose urinary protein contains mixtures of high and low
molecular weights.
Proteinuria will often start out heavy
when the disease first appears. Like hematuria, it can be
exacerbated by respiratory or other illnesses, or even by
emotional stress.
If protein loss rises to heavy levels
during the course of IgAN and remains high, this is
usually as sign of a poor prognosis. Heavy proteinuria
often precedes the development of hypertension (see
below). When protein loss is massive (usually over 3
g/day), it can provoke nephrotic syndrome,
symptoms of which include edema, fatigue, anorexia
[unwillingness to eat], abdominal pain, and wasting of
muscles. If left untreated, heavy urinary loss of certain
proteins, as well as of immunoglobulins and hormones, can
lead to nutritional deficiencies, bone demineralization,
and a high incidence of infections.
^^^top^^^
What
other side effects or symptoms does IgA Nephropathy have?
The dominant complaint of most patients
is of fatigue, which can be overwhelming. They may
have a sore throat (which should be tested for strep), or
a headache, or some other problem, but none of the overt
symptoms is sufficiently grave to explain how bad they
feel or how tired they are. Exhaustion can make it
difficult, even impossible, to concentrate, do schoolwork,
or perform their job. Episodes of apparent illness
combined with fatigue can last for several days or more.
There is no way to predict when or how often they will
strike or how long they will last. During these episodes,
patients may have a peculiarly strong and foul breath,
loss of appetite, dry skin, pallor, weakness, inability to
concentrate, and shortness of breath.
Unfortunately, IgAN’s side effects are
not well documented in the medical literature, so many
nephrologists are unfamiliar with many of the problems the
disease can cause. One authority notes that there are few
apparent symptoms in the disease, although among those
that do occur during episodes of gross hematuria are malaise
[feeling of weakness and lethargy], low-grade fever,
flulike symptoms, flank pain, and myalgias
[pain in the muscles]. Patients report, however, that
these symptoms can occur without hematuria.
Episodes of gross hematuria and rises in
proteinuria often seem triggered by colds or other
respiratory infections, or even by lack of sleep. At other
times there is no apparent trigger. Some patients have
sore throats and headaches as if coming down with a cold,
yet they have no fevers and tests for bacteria are
negative, suggesting that a virus — or an autoimmune
reaction — may be involved. Emotional stress may also
aggravate proteinuria, at least temporarily. (Even
hereditary disorders like hemophilia have their symptoms
exacerbated by stress.)
It may look as if the patient is
immune-deficient and falling prey to every bug that comes
along, yet full-blown symptoms of respiratory illness
(sneezing, runny nose, cough) rarely develop. IgAN seems
to follow a seasonal pattern, worsening in winter
and spring. Patients report an increased number of
"colds" then, but it is unclear if these are
actual viral/bacterial infections or hyperimmune
reactions. Many also report that it takes them a month or
more to get over colds that other family members shake off
in days.
In general, patients with IgAN seem to need
much more sleep (12 hours or more a day) than most
people. Most patients find that they tire more easily and
have much less stamina than formerly. We have had reports
of children losing sensitivity to temperature change
so they do not feel the cold and tend to underdress; this
has not yet been confirmed in adults. Developing allergies
or contrary reactions to medications is more common
among both children and adults. Some patients seem to go
from wellness to illness within as little as an hour or
less, as if experiencing an allergic reaction.
Many patients have bouts of flank
pain, which can range from a dull ache in the back
(often confused with lower back problems or arthritis) to
debilitating spasms that make walking and rising
difficult. Hypertension [high blood
pressure] is not usually found when IgAN is first
diagnosed, but it often develops in the course of the
disease. Because of the kidneys’ important role in
controlling blood pressure (by releasing enzymes that tell
blood vessels to relax or constrict and by excreting
excess salt), hypertension is a frequent complication of
most kidney disorders. Hyperlipidemia
[excessive fat lipids in the blood] and
elevated cholesterol levels are also very common in
diseases that, like IgAN, involve immune reactions.
Some patients, especially children,
experience occasional excruciating abdominal pain
that may be mistaken for appendicitis. Unlike
appendicitis, however, the white blood cell count is not
elevated, there is no fever, and an ultrasound scan of the
abdomen, while painful, may show only swollen lymph nodes.
This abdominal pain has also been mistaken for renal
colic [severe pain produced by passage of a kidney
stone], or, in women, a ruptured ovarian cyst.
You may notice mood swings:
patients with IgAN can become hyper-irritable, querulous,
demanding, although within a few days, as their strength
returns, so does their sense of humor and their good
nature. When feeling ill, they can become very pale, with large,
dark circles under the eyes. Some also have periorbital
edema [swelling from retained fluid around the
eyes] in the morning and swelling of the ankles in the
evening.
Because IgAN so often seems to appear
after a series of seemingly unrelated illnesses (for
example, pneumonia, mononucleosis, strep throat,
middle-ear infections, etc.), an underlying viral
infection may be present. Allergic reactions may also
complicate IgAN and be reflected in some of its symptoms.
Many IgAN patients seem prone to developing itchy,
hives-like rashes [urticaria] over large
areas of their bodies. The cause of this is not known, but
it may be associated with deposition of immunoglobulin A
in the skin.
^^^top^^^
If
I experience flank pain, how should I treat it?
Flank pain often starts as a little
discomfort in the area above one or both kidneys. It may
feel as if a nerve is pinched in that area, and the skin
over the kidneys may be very sensitive to the touch. If
the area over the kidneys is sore — but not the area
over the spine — you should suspect flank pain.
There are no good medical treatments for
flank pain. Aspirin can aggravate bouts of gross hematuria,
and doctors warn against using it in children and
teenagers whenever there is a possibility of a viral
infection because of the danger of developing Reye’s
Syndrome. Acetaminophen (Tylenol) and ibuprofen (Motrin,
Advil) are both nephrotoxic and should not
be taken in quantity for any type of pain, including
headaches. Besides, painkillers appear to have little
effect on flank pain. Similarly, sedatives and
muscle-relaxants like Valium do little once an attack is
underway, nor is there evidence that such heavy-duty
painkillers as Demerol or nerve blocks are effective.
Hypnotherapy, biofeedback, and
acupuncture are useful in managing certain types of pain,
but there is no information yet on whether any of these
works with flank pain. Homeopathy is most likely to offer
symptomatic relief that is also safe and non-toxic.
The simplest approach to handling flank
pain is to catch it at the start and apply heat to the
area with a hot water bottle, heating pad, or soaks in a
warm bath or shower. Hot castor oil packs over the
affected area may be helpful, if messy.
Try to increase fluid intake during
attacks. If you have frequent attacks, especially of
severe spasms of pain, watch your morning urine specimen
carefully. If it is turbid [very cloudy] with a fine,
white sediment, consider taking yourself off ALL dairy,
ALL meat products, and ALL sodas until the urine clears
and the pain ends. Stay away from refined sugar; use lots
of fresh fruit and vegetables and only WHOLE grains. We
have no proof that this regimen works for all, but it is
worth a try if you are in severe pain.
It’s also a good idea to stay away
from caffeine, alcohol, and tobacco during any
exacerbations of the disease. The Network has also
received reports that repeated bending and overuse of the
back may aggravate flank pain. If this is a feature of
yours work, you may need to speak to your employer about
possible modifications to your job.
As with so much of IgAN, we do not know
what causes flank pain, and not knowing the cause makes
treatment largely a matter of experimentation. If you’ve
found something that works for you, we welcome your
suggestions.
^^^top^^^
What
happens to the kidneys during the course of this disease?
IgAN was thought relatively benign when
it was first identified in 1968 by the French doctor, Jean
Berger, but gradually researchers realized that in up to
50% of the cases, the disease very slowly progresses to
end-stage renal failure, requiring major lifestyle
changes, dialysis, and possibly transplantation.
IgAN does not immediately interfere with
the kidneys’ filtration work — except in acute cases
— but it may threaten it ultimately. Damage results as
kidney tissue gradually develops irreversible scarring,
which ultimately blocks renal capillaries, causing the
death of surrounding tissues as these are deprived of
blood and nutrients. The slow accumulation of these tiny
affected areas can take ten or twenty — or more —
years to produce kidney failure, although a small minority
of patients, mainly adults, have a rapidly progressive
form that results in renal failure within a few years or
even months.
As individual capillaries become clogged
by coagulation excessive cell growth, or infiltration by
inflammatory cells, the nephron loses filtering surface.
The kidneys’ glomerular filtration rate [GFR],
a measure of how efficiently the kidneys filter blood,
declines when that loss is enough to overcome the body’s
efforts to adapt by increasing pressure throughout the
remaining nephrons. As GFR decreases, the kidneys nose
their ability to cleanse the blood of toxins and metabolic
byproducts. Kidney failure and uremic poisoning result.
It is difficult to predict from initial
clinical signs and biopsy results just which patients will
have which course. Statistical analyses have yielding
conflicting results, but some factors do seem linked to a
poor prognosis. Unremittingly heavy proteinuria,
uncontrolled hypertension, declining glomerular filtration
rate, high serum creatinine, and being age 30 or older at
the time of apparent onset of the disease are some of the
factors tied to a poor prognosis. Severe lesions in the
initial biopsy, especially evidence of sclerosis
[scarring] or crescent formation are also
ominous signs.
^^^top^^^
How
will the doctor treat this disease?
There is no proven treatment for IgAN.
Your doctor’s recommendation as to whether to treat or
not probably will be based on three factors:
1. The severity of the lesions, as
revealed by the biopsy;
2. The degree of proteinuria (usually
proteinuria greater than 2,000 mg/day warrants treatment);
3. The creatinine clearance (if less
than 70 ml/min [milliliters per minute],
treatment is generally recommended).
Doctors usually try to curb the acute
inflammation signaled by heavy proteinuria. In severe
cases, some employ high intravenous doses of methylprednisolone,
a glucocorticoid that is anti-inflammatory and
immunosuppressive. This is called "pulse
therapy," and it is usually employed for only short
periods. In less acute cases, most physicians use oral
corticosteroids, like prednisone, which are also
anti-inflammatory and which help to stabilize the
glomerular basement membrane, making it less permeable to
large protein molecules. Prednisone is given either
daily or every other day to minimize its toxic side
effects.
Unfortunately, prednisone does not work
in all cases, not is there any proof that it does much
more than temporarily slow the disease process. Prednisone
(or any corticosteroids) has potentially serious side
effects when used long-term (e.g., bone damage and necrosis
[tissue death], weakening of arm and leg muscles, peptic
ulcers, diabetes or hyperglycemia, increased
susceptibility to infection, cataracts, acne, weight gain,
manic or even psychotic behavior, and lesser
"cosmetic changes" such as a moon face). Its use
needs to be thoroughly discussed with your nephrologist.
Some doctors report that patients on prednisone seem to
feel better than untreated patients, perhaps because
prednisone makes them feel "up" and energized.
On the other hand, some believe that using prednisone
reduces chances of having a natural remission.
Because immunosuppressants can
reactivate even dormant viruses, transplant candidates are
routinely tested for viruses. But IgAN patients in the
early stages of the disease are not. Before going on
prednisone or any other drug that suppresses the immune
system, it is vital that you be tested for tuberculosis
and for the possibility of a viral infection. Rather
than testing for individual viruses such as Epstein-Barr (EBV),
cytomegalovirus (CMV), or various herpes viruses, your
doctor may want to order an immunoradiometric assay
of alpha-interferon levels in your. An antiviral
glycoprotein, alpha-interferon is usually undetectable in
the blood of normal individuals; its presence, usually in
high concentrations, is a fairly reliable indicator of a
viral infection. Also inform your doctor if you’ve never
had such diseases as measles or chickenpox or mumps.
Immunosuppressive drugs can turn a mild childhood disease
into a major threat.
Other treatments than prednisone, based
on other rationales, have been tried with little or no
success. The anti-convulsive drug phenytoin (Dilantin)
was found to lower levels of circulating IgA in the blood,
but did nothing to alter the course of the disease.
Anti-coagulants like warfarin and platelet inhibitors like
dipyridamole have been employed, usually in
combination with other drugs, because excessive clumping
of blood platelets in the glomerular capillaries is
suspected of being an accessory in damaging the kidneys. A
gluten-free diet, adopted to remove food allergens
that might be triggering the body’s immune response, had
some success but was difficult for many to follow. More
recently, a low-antigen diet has shown promise.
[See Should I follow a special diet?]
Cytoxic drugs
used in chemotherapy, like cyclophosphamide (Cytoxan), and
drugs used to prevent organ rejection in transplants, such
as azathioprine (Imuran) and cyclosporine (Sandimmune),
are powerful immune suppressants that carry unacceptable
risks for all but the most relentless cases.
Unfortunately, the Network has received a number of
reports over the years of such drugs being used on
children without their parents being fully informed of the
potential consequences, such as sterility. If you are not
informed of ALL potential side effects of a treatment,
there is no such thing as "informed consent."
Plasmapheresis
[a process in which such blood constituents as red blood
cells are separated from the plasma and returned to a
patient’s system] is widely used in Japan to treat
immune-complex mediated diseases like IgAN, but doctors
here question its usefulness. Removing immunoglobulins
from the blood makes a patient more vulnerable to serious
illness. It also removes clotting factor, raising the risk
if hemorrhage in the event of injury. On the other hand,
in cases of rapidly progressive IgAN, combinations of
steroids, cytoxic drugs, and plasmapheresis may be
helpful, at least temporarily.
Another treatment that has enjoyed a
certain vogue is the tonsillectomy. This may help
those who are prone to sore throats and infected tonsils,
but there is no proof it affects the course of IgAN.
Chronically infected tonsils can also be treated by
non-surgical means, such as homeopathy.
You should discuss with your doctor ALL
the potential side effects of any therapy proposed and
carefully weight possible risks against possible benefits.
There is no real proof that any currently used medical
therapy is of any value in treating IgAN, at least among
those who do not have the rapidly progressive form.
Some have shown promise in ameliorating symptoms. None is
curative.
In relatively mild cases of IgAN, your
doctor may recommend treatment with fish oil high
in eicosapentanoic acid [EPA],
which has anti-inflammatory properties and which may also
lower plasma triglycerides and cholesterol. Results have
been mixed, but many doctors believe this can be an
effective anti-inflammatory if started early. [See Fish Oil] Fish oil is unlikely to have harmful side
effects, but it does have anti-coagulant properties that
can exacerbate heavy cases of gross hematuria. Of greater
concern is the possibility of heavy metal or dioxin
contamination, especially at the "standard"
adult dosage of a whopping 12 g/day. You might wish to
consider starting with a substantially smaller dose, such
as 3 g/day, to see if produces the desired effects of
reducing inflammation and proteinuria. Another possibility
is to use flaxseed oil or perilla oil as
alternatives. From vegetable sources, these oils are free
of heavy metal or dioxin contamination, are high in EPA,
and are more easily tolerated than fish oil, although you
may need to take more of them to acquire the same
concentrations of EPA. (A recent Consumer Reports survey
of fish oil capsules found none that contained
"significant" amounts of mercury, PCBs, or
dioxin.)
Because it is vital to treat
hypertension if that is present initially or develops,
your doctor may prescribe anti-hypertensive medication.
Uncontrolled hypertension is strongly linked to more rapid
progression of IgAN, perhaps because excessive pressures
within the kidney strain already damaged glomerular
capillaries. Hypertension can often be managed
successfully through weight loss, low-fat diet, and
regular aerobic exercise; in kidney disease, however, your
doctor may have to try several medications before finding
the right one.
Diuretics are perhaps the least
effective in controlling hypertension associated with
renal disease. ACE inhibitors like enalapril (Vasotec)
or ramipril (Altace) or beta-blockers are usually a better
choice. They have been found to reduce the risk of kidney
failure when compared to calcium channel blockers also
used to control hypertension.
Studies suggest that ACE inhibitors, by
inhibiting the formation of the hormone angiotensin, help
preserve the integrity of the glomerular membranes, making
them less permeable and thereby decreasing proteinuria.
They may also lessen the scarring of kidney tissue by
interfering with the process that stimulates mesangial
proliferation [excessive growth of mesangial
cells]. Many doctors use ACE. inhibitors prophylactically,
administering small doses even before hypertension
appears. Dosage must be carefully monitored to avoid hypotension
[extremely low blood pressure] and dizziness. The
long-term effects of using ACE inhibitors in children are
not clear. Patients may develop a chronic cough on the
medication and have to switch to another anti-hypertensive
drug.
Hyperlipidemia is also associated with
renal disease, and your doctor may wish to treat that with
dietary changes or cholesterol-lowering drugs. It is
important to reduce cholesterol levels to prevent heart
disease; moreover, high cholesterol levels may aid the
progression of IgAN. When lipids accumulate in the
kidneys, possibly following injury to the mesangium, they
are believed to contribute to scarring of the glomeruli.
Again, treatment is a topic to be thoroughly discussed
with your doctor. Cholesterol-lowering drugs, such as
statins, can have dangerous side effects, including liver
damage, memory loss, and rhabdomyolysis [a
potentially fatal disease marked by destruction of
skeletal muscle].
For a detailed discussion of statin
drugs and other traditional means of lowering cholesterol,
as well as alternatives to these, see the Life Extension
Foundation, "Cholesterol Reduction" ( http://www.lef.org/protocols/prtcl-032.shtml
). The Life Extension Foundation sells nutritional
supplements, but their research is thorough and reputable.
Redflagsdaily.com, a health-oriented website started by
Nicholas Regush, a Canadian journalist specializing in
medical and scientific issues, has also explored problems
with statins and other drugs; but to access this material
requires subscribing the site.
^^^top^^^
Should
I follow a special diet?
Your doctor may want to put you on a low-salt
diet to minimize stress of the kidneys and prevent
excess fluid retention that would strain your heart. This
is especially likely if you have shown signs of edema or
hypertension. Even if you are not showing such signs, a
low-sodium diet may be a good idea. It won’t hurt and it
may well help, particularly for those being treated with
prednisone, which tens to make the body retain sodium. How
strict a diet is the question.
When we think "low sodium," we
think of eliminating potato chips, pretzels, cocktail
peanuts — all snack foods with added table salt (sodium
chloride). But sodium appears in other forms, such as
sodium bicarbonate (baking soda), sodium propionate (a
preservative), dihydroxyaluminum sodium carbonate
(antacids), sodium fluoride (toothpaste), and monosodium
glutamate (m.s.g.). Whether the culprit is all forms of
sodium or only table salt is not clear. Sodium chloride is
naturally high in some foods, from soup to pickles to
breakfast cereals. Even some frozen vegetables, such as
peas and lima beans, have added salt.
Whether your low-salt diet is very
strict (no more than 1,000 mg of sodium per day), or more
lenient (2,000 mg per day), you will probably have to
eliminate certain favorites, namely, all fast foods,
pizza, cheesesteaks, hoagies, prepared snacks, etc. Plan
on becoming an avid label-reader. Health food stores and
most supermarkets offer low-salt or no-salt substitutes
for some items (catsup, mayonnaise, deli meats and
cheeses, salad dressings, crackers, cookies, potato and
taco chips, soups, candies, etc.). They also offer
assortments of herbs that can be used in lieu of salt (not
to be confused with commercial salt substitutes that rely
on potassium in place of sodium; these are not
recommended).
You may find it easier to put the entire
family on a low-salt regime, so the patient doesn’t feel
"left out." It’s healthier for everyone, but
it does take cooperation on the family’s part and
flexibility on the cook’s. Paradoxically, the new diet
may be more easily accepted if you radically revamp your
cooking style rather than trying to remake old favorites
or introduce low-sodium analogs to familiar foods. Ethnic
cuisines that rely heavily on herbs or citrus juices —
for example, Italian, Mexican, and Thai — are more
easily adapted to low-salt cooking.
Another possibility is to adopt a
basically vegetarian diet. Those with moderate to heavy
proteinuria should strongly consider eliminating meat
and milk products, basing their diet upon fresh fruits
and vegetables, whole grains, and such vegetable sources
of protein as nuts, beans, legumes, soy, and fish, while
avoiding processed or fast foods. This type of diet saves
the kidneys a lot of hard work in clearing the body of the
byproducts of metabolizing animal proteins. There are also
chemicals in milk and meat that exacerbate the
inflammatory process taking place in the kidneys.
There are no cookbooks specifically for
IgAN patients, but there are many fine vegetarian as well
as low-salt cookbooks available. [For a list of
recommended ones, see Vegetarian Cookbooks]
When evaluating cookbooks, it is important to use ones
that do NOT rely on dairy products as a substitute for
meat.
In the later stages of kidney failure,
patients are put on very restricted diets. There are
various types, depending upon how much renal function has
been lost, but most limit protein and potassium intake.
Some doctors believe a low-protein diet should be adopted
much earlier to spare the kidneys and slow down the
disease process, while others advocate eliminating dairy
products because milk proteins irritate the immune system.
It used to be thought that a low-protein
diet worked to preserve kidney function by reducing
pressures within the glomeruli, but recent animal
experiments suggest that it helps suppress the expansion
of extracellular matrix that occurs in glomerulonephritis
— and that is implicated in the scarring of the
glomeruli. A diet based on vegetable proteins from beans,
lentils, grains, and nuts is rich in "good"
fatty acids, which can help dampen the inflammatory
response that damages the kidneys in IgAN. [For more
information, see Not What We Expected.
Often patients with IgAN have multiple
allergies or a family history of other immunologically-mediated
problems, such as asthma. An Italian study reported a
marked reduction in proteinuria in IgAN patients put on a
strict low-antigen diet [a diet free of
foods likely to cause an allergic reaction]. Such diets,
which have also proved effective in treating
Attention-Deficit Disorder, may exclude such commonly
allergenic foods as wheat, corn, soy, food preservatives,
and food coloring agents. Excluding allergenic foods has
become more difficult in recent years because of the rise
of genetically-modified foods that may contain foreign
proteins capable of triggering an allergic reaction. For
highly sensitive people, eating certified organic foods
may be the only way to avoid genetically-modified
foodstuffs.
Food allergies can be difficult to
detect by observation, because people may have a delayed
reaction to — or even a craving for — foods to which
they are allergic. The least expensive way to approach
this problem is to go on an elimination diet in which
allergenic foods are removed, then slowly rotated back
into the diet, one at a time. A more expensive, but also
more thorough approach, is to be tested for delayed
hypersensivity reaction through an ELISA/ACT test.
[For more information, see Dampening the Inflammatory Response. ]
^^^top^^^
What
could I be doing to help myself?
This is a highly personal issue. Often
patients feel frustrated because there is so little that
can be done for people with IgAN, particularly in the
early stages of the disease. We have actually had patients
report that their doctors told them: "Come back when
you need dialysis, because there is nothing I can do for
you now." Needless to say, that approach doesn’t
help anyone who’s suffering from a difficult and poorly
understood disease!
The Network suggests a number of ways
you may help manage your IgAN. Not all of these will
benefit everyone, because our responses to therapies, as
to disease, are conditioned by individual differences. But
as long as there is no medical cure for IgAN, it is worth
checking out non-drug means of slowing the disease’s
progression.
1. Experiment with dietary changes,
as recommended above. It can be tricky to persuade
teenagers to sacrifice fast food for the sake of their
kidneys, but sometimes they’re willing to do a trial of
a new diet for a month or two to see if it makes them feel
better.
2. Avoid tobacco.
Nicotine constricts the blood vessels, raising pressure
and affecting peripheral circulation. People with renal
disease don’t need anything that may induce higher blood
pressure or impair circulation.
3. Drink plenty of pure water.
If you are not absolutely sure about the purity of your
municipal or private water supply, drink distilled water
(available at drugstores and some supermarkets). Avoid
sodas, like colas, that are high in phosphoric acid,
especially if you have suffered some loss of kidney
function.
4. Support your liver.
To give your liver as much help as possible in its work of
removing immune complexes from the body, reduce or
eliminate your intake of caffeine, which is found in many
sodas, cocoa, chocolate, and tea as well as coffee. Use
alcohol in moderation; excessive drinking may aggravate
the disease. Naturopaths also recommend use of supportive
herbal therapies, such as extracts of dandelion root
and/or milk thistle to help cleanse the liver.
5. Avoid medications toxic to the
kidneys. This includes such
painkillers as Tylenol, Advil, Motrin, etc. All of these
contain either ibuprofen or acetaminophen, both of which
are nephrotoxic. The same is true of most prescription
painkillers. [For more information, see Headaches
and Analgesics] Whenever you are treated
by a doctor other than your nephrologist, be sure that
doctor is aware of your IgAN and knowledgeable about not
prescribing any medication or antibiotics (such as
tetracycline) that might be toxic to your kidneys, either
by themselves or in combination with other drugs. Toluene,
either through industrial exposure or glue-sniffing, is
also toxic to the kidneys.
6. Take anti-oxidants.
Vitamin C and vitamin E, pycnogenol or grapeseed extract
can be very helpful. Injury to the kidneys is largely
caused by free radicals released as part of the body’s
inflammatory response; anti-oxidants act as scavengers to
remove these from the body, thereby mitigating the damage.
[For other supplementation that may be helpful,
particularly in more advanced disease, see Life Extension
Foundation, Disease Prevention and Treatment, 4th
edition (2003), "Kidney Disease," pp. 963-78.]
7. Look to alternative medicine.
There is much that homeopathy, naturopathy, Chinese herbal
medicine, and acupuncture can do to alleviate specific
symptoms as well as retard the progress of IgAN,
particularly in the early stages. It is our belief that
BEFORE employing drastic medical treatments, such as the
use of immunosuppressive drugs, gentler, non-toxic means
should be considered. The difficulty is finding a
practitioner who is skilled in treating not necessarily
IgAN but other complex, chronic, immunologically-mediated
conditions such as rheumatoid arthritis, fibromyalgia,
chronic fatigue syndrome, and lupus. [See Links
to Homeopathic Organizations for Referrals]
8. Be comfortable with your doctor.
This is more difficult to achieve in these days of managed
care, but be sure you are working with a nephrologist you
trust, one who answers your questions fully and freely. If
you have any doubts about your doctor’s competence or
availability, start looking for another nephrologist. This
is a long-term relationship; it should be one you feel
good about. If you are not in need of dialysis and hope to
avoid it in the future, you might want to steer clear of
nephrologists who have a financial interest in dialysis
centers. At its worst, this is a blatant conflict of
interest; at best, it raises the question of whether such
physicians are focused on preventing the progression of
renal disease.
9. Get plenty of sleep.
Most IgAN patients complain of fatigue and need more sleep
than before they became ill.
10. Watch your physical activities.
Exercise is a good idea, and it is rare to have any
limitations put on it. But it doesn’t hurt to keep an
eye on how strenuous your activities are, particularly if
they seem to be inducing hematuria.
11. Test your urine. This
suggestion is most likely to appeal to parents who want to
monitor their children’s proteinuria and hematuria. Some
patients find it reassuring to test and look for patterns
in their proteinuria or hematuria; others may feel more
comfortable sticking with the 24-hour urinalyses
prescribed by their doctor. Chemically-treated dipsticks
are available for different kinds of tests. Ames Albustix
test only for urinary protein, whereas their Multistix
test not only for protein but also for blood, specific
gravity, pH, ketones, leukocytes, etc. The latter are
expensive, but useful when it comes to checking on
hematuria. Your druggist can order these for you. (Note:
Dipstick tests measure only the concentration of protein
in a given sample, usually the first morning void. They
won’t tell you how much protein you’re losing on a
given day — only a 24-hour urinalysis can do that.)
12. Avoid stresses to your immune
system. Because the immune
system is not operating properly in IgAN, it is important
to avoid placing unnecessary stresses upon it. You should
minimize your exposure to things to which you are allergic
(or know you are allergic) and avoid people who have
coughs and colds, especially during flu season. (This
advice is hard to follow for teachers and parents with
little ones.) Vaccinations are another major source of
stress to the immune system; indeed, there are cases of
IgAN that developed following vaccination, particularly
when more than one vaccination was given or the person was
vaccinated when ill, as can happen in the military. It is
a good idea to avoid vaccinations that are not absolutely
necessary and to avoid all multivalent vaccines. Space
inoculations as far apart as possible — one week at
least — and take plenty of vitamin C afterward. If your
child has IgAN, talk to his or her doctor about getting an
exemption from state-mandated vaccinations on grounds of
health. If you are worried about contracting influenza,
whooping cough, mumps, or any of the other diseases
covered by vaccines, talk to a homeopath or naturopath
about alternatives to vaccination. [For more information,
see To Vaccinate or Not]
13. Keep in mind that IgAN is a CHRONIC
disease. It’s not like a bout
of bronchitis — there’s no pill that can cure it. It
is not a disease that suddenly and spontaneously remits.
Overcoming IgAN takes not weeks but years. You can expect
to be sick much of the time, particularly in cold weather.
Dealing with chronic illness requires a lot of support,
for you and your family, because it is an emotionally (and
financially) draining experience. Unfortunately, given
IgAN’s relative rarity, you are unlikely to find a
support group devoted to this disease, but you may be
able, with the help of your hospital’s nephrology
department, to put together a group based on kidney
disorders in general. The diseases may be different, but
you’ll face a lot of common problems. If you are
married, your spouse may wish to join a caregivers’
support group, which some hospitals offer. Remember, too,
that because IgAN is chronic, you’ll have to be followed
regularly by your doctors. Even if you are asymptomatic
[showing no symptoms of the disease], it is important to
have periodic evaluations in accordance with your
nephrologist’s recommendations. Sometimes the disease
can worsen even though you are showing no overt symptoms.
^^^top^^^
What
are my chances of having a remission without treatment?
It is impossible to say, because this
has not been well researched. While it is extremely
unlikely for adults to undergo spontaneous remission,
remissions do appear to occur occasionally among children.
One study estimated that in perhaps 4% of reported IgAN
cases there had been a spontaneous remission of all
clinical symptoms. Whether this was paralleled by the
spontaneous disappearance of IgA deposits in the kidneys,
or by the restoration of normal glomerular structure is
unknown. Patients in remission are rarely biopsied or
studied to see what might have induced their remission.
Some doctors believe the use of
corticosteroids or other immunosuppressive medications may
reduce chances for a natural remission.
^^^top^^^
What
happens to those who go on to kidney failure?
Except for the very few who have rapidly
progressive IgAN, most patients will take years, even
decades, to develop kidney failure. Those who have end-stage
renal disease [ESRD] are treated
with dialysis and may be candidates for transplantation.
Deposits of IgA, the hallmark of the disease, frequently
appear in transplanted kidneys, but the clinical symptoms
do not usually come back. An early survey of transplants
due to IgAN showed that although IgAN-type lesions
recurred in over 50% of the respondents, only 3% lost
their new kidneys to a resurgence of the disease.
More recently, doctors have observed
deposits of immunoglobulin A in up to 80% of patients who
have received transplants after their own kidneys were
destroyed by IgAN; yet only one quarter of these showed
any clinical symptoms. About 20% of those transplanted
lose their grafts to recurrent IgAN. According to one
nephrologist who has followed this subject, graft failure
came generally after 8-10 years, which would be the
expected lifespan for a cadaver kidney anyway. In other
words, recurrent IgAN did not contribute to early,
unexpected loss of a transplant.
The possibility of recurrent IgAN should
not discourage patients from having a transplant, but they
should be aware that a transplant is not a cure for the
disease, merely a replacement organ.
^^^top^^^
Will
my children develop IgAN?
IgAN is not a hereditary disease, like
cystic fibrosis or Huntington’s chorea. In certain
families, there does appear to be a predisposition to
develop the disease, suggesting a genetic component. But
before you can develop IgA Nephropathy, there must be some
kind of trigger. We just don’t know what that is.
If you are worried about your children
possibly developing IgAN, make sure to have their urine
tested as part of a routine physical exam. Urinary
abnormalities are fairly common, so don’t assume the
child has IgAN if a single test shows a problem. That’s
just a signal that more thorough testing is necessary.
Occasionally the Network has encountered
families with two or more cases of IgAN, but in our
experience this has generally been sex-linked (e.g.,
mother-daughter, uncle-nephew).
^^^top^^^
Should
a woman who has IgAN bear children?
This is a tough call and one that can be
made only on an individual basis, after careful
consultation with your nephrologist. You may not have to
worry about transmitting it to your unborn child (it’s
not communicable to a fetus like AIDS), but you do have to
worry about whether IgAN will affect your baby in other
ways and whether pregnancy might accelerate progression of
the disease in you.
Studies generally agree that women who
do NOT have hypertension or impaired renal function have a
very good chance of producing a healthy infant, with no
adverse effects to their own health. According to
guidelines published by one researcher, this means that
your blood pressure must consistently be under 140/90 mm
Hg and your serum creatinine no more than 1.1. mg/dL
(equivalent to a glomerular filtration rate of 70 ml/min
or better) before you become pregnant. Hypertension may be
brought on by pregnancy, but if it can be
controlled by medication it should pose no threat to the
fetus.
Women with chronic glomerulonephritis,
such as IgAN, do run a higher risk of miscarrying, of
having a stillborn baby or one with low birth weight and
growth retardation, and of having complications during
birth, such as premature delivery or severe bleeding after
delivery. One Japanese study of various kidney diseases
found that 90% of the pregnancies in women with normal
blood pressure were successful, whereas hypertensive women
lost their babies 33% of the time. Some centers report
even higher loss rates, but that may be due to having
patients with more numerous complications. We have talked
to women who lost as many as five pregnancies before their
underlying IgAN was discovered.
Women with kidney disease run a
heightened risk of developing preeclampsia,
a serous condition that can appear late in pregnancy and
is characterized by a sudden rise in blood pressure,
excessive weight gain, edema, proteinuria, terrible
headaches, and visual disturbances. Preeclampsia threatens
both mother and fetus.
There is also the possibility that
pregnancy may worsen a woman’s IgAN. Again, generally
speaking, the risks are greater for a woman whose blood
pressure is high or whose kidney function already impaired
at the beginning of her pregnancy. There are a minority,
however, who start with good clinical signs but rapidly
deteriorate during pregnancy or after delivery. On their
biopsies, some researchers find, these women are likely to
have tubulointerstitial changes involving
more than 20% of the renal cortex [outer
layer of the kidney]. They may also have arteriolosclerosis
[thickening of the lining of the kidney’s
arterioles, which are the terminal branches of its
arteries].
To assess how risky a pregnancy might be
for you, you should discuss your biopsy and lab results
with your nephrologist, preferably before becoming
pregnant. Another factor to consider is the availability
of specialized obstetrical care in your area. Women with
IgAN are likely to require the services of an obstetrician
who is willing and qualified to handle high-risk
pregnancies.
^^^top^^^
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