Wegener granumalatosis

By meghna banerjee
• What is granulomatosis with polyangiitis (GPA, formerly called
Wegener’s)?
• Granulomatosis with polyangiitis (GPA, formerly called Wegener’s) is a rare
disease of uncertain cause. It is the result of inflammation within the tissues
called granulomatous inflammation and blood vessel inflammation
("vasculitis"), which can damage organ systems. The areas most commonly
affected by GPA include the sinuses, lungs, and kidneys, but any site can be
affected
First Description
The first case was described by Heinz Klinger, a
German medical student, in 1931. Several years
later a German pathologist, Friedrich Wegener,
described 3 additional cases and recognized the
disorder as a distinct form of vasculitis. Other
names occasionally used for Granulomatosis
with Polyangiitis are Wegener’s
arteritis or Wegener’s disease.
• Causes
• The cause of granulomatosis with polyangiitis isn't known. It's not
contagious, and there's no evidence that it's inherited.
• The condition can lead to inflamed, narrowed blood vessels and harmful
inflammatory tissue masses (granulomas). Granulomas can destroy normal
tissue, and narrowed blood vessels reduce the amount of blood and
oxygen that reaches your body's tissues and organs.
• Pictured below are two figures that show subglottic stenosis before (left)
and after (right) surgery, performed by an Ear, Nose, & Throat specialist.
The surgery provided dramatic improvement in the patient’s breathing.
• Complications
• Besides affecting your nose, sinuses, throat, lungs and kidneys, granulomatosis
with polyangiitis can affect your skin, eyes, ears, heart and other organs.
Complications might include:
• Hearing loss
• Skin scarring
• Kidney damage
• A loss of height in the bridge of the nose (saddling) caused by weakened
cartilage
• A blood clot forming in one or more deep veins, usually in your leg
The image below is from a urinalysis of a patient with kidney inflammation.
When Granulomatosis with Polyangiitis is active, red blood cells will form
a clump or “cast” (bracketed in white) within the tubules of inflamed
kidneys. These “casts” pass through the renal system and may be viewed
under the microscope in a patient’s urine.
First Description
The first case was described by Heinz Klinger, a German medical student,
in 1931. Several years later a German pathologist, Friedrich Wegener,
described 3 additional cases and recognized the disorder as a distinct
form of vasculitis. Other names occasionally used for Granulomatosis with
Polyangiitis are Wegener’s arteritis or Wegener’s disease.
Who gets Granulomatosis with Polyangiitis?
Granulomatosis with Polyangiitis is nearly equally distributed between the
sexes, with a slight male predominance. Granulomatosis with Polyangiitis
typically occurs in middle age, but is found in people of all ages. Although
it is unusual for Granulomatosis with Polyangiitis to occur in childhood, it
is not unusual for a Granulomatosis with Polyangiitis patient to be in his/
her 70s or even 80s at the time of diagnosis.
Pictured below is a chest x–ray showing bilateral lung nodules in a 27
year old Indian man with Granulomatosis with Polyangiitis.
• Urine tests can reveal whether your urine contains red blood cells or has
too much protein, which might indicate that the disease is affecting your
kidneys.
• Imaging tests
• Chest X-rays, CT or MRI can help determine which blood vessels and
organs are affected. They can also help your doctor monitor whether
you're responding to treatment.
• Biopsy
• This is a surgical procedure in which your doctor removes a small
sample of tissue from the affected area of your body. A biopsy can
confirm a diagnosis of granulomatosis with polyangiitis.
• More Information
Pictured below is a computed
tomography (CAT) scan of the
eyes in a patient with a
retro–orbital mass (a mass
behind the eye) in a man with
Granulomatosis with
Polyangiitis. Masses such as
these sometimes cause an
abrupt loss of vision through
stretching or compression of
the optic nerve, which enters
the back of the eye.
Pictured below is a chest x–ray showing
bilateral lung nodules in a 27 year old Indian
man with Granulomatosis with Polyangiitis.
Pictured below is a CT scan from the same
patient. The view is a cross–section through the
patient’s lungs. The CT scan not only permits a
better appreciation of the lesions’ size, it also
detects more lesions
 chest x–ray showing bilateral lung nodules in a 27 year old
Indian man with Granulomatosis with Polyangiitis.
• Pictured below is a CT scan from the same patient. The view
is a cross–section through the patient’s lungs. The CT scan not
only permits a better appreciation of the lesions’ size, it also
detects more lesions.
• Granulomatosis
• Ear infections that are slow to resolve. Recurrent otitis media.
Decrease in hearing.
• Eye
• Inflammation can occur in different parts of the eye. Inflammation in the white part of the eye is known as the sclera (“scleritis”). “Uveitis” is inflammation within the eye.
Inflammation behind the eye is known as an “orbital pseudotumor”. An orbital pseudotumor such as those caused by Granulomatosis with Polyangiitis can cause
“proptosis”, or protrusion of one eye.
• Pictured below is a computed tomography (CAT) scan of the eyes in a patient with a retro–orbital mass (a mass behind the eye) in a man with Granulomatosis with
Polyangiitis. Masses such as these sometimes cause an abrupt loss of vision through stretching or compression of the optic nerve, which enters the back of the eye.
• Nose
• Nasal crusting and frequent nosebleeds can occur. Erosion and perforation of the nasal septum. The bridge of the nose can collapse resulting in a “saddle–nose
deformity”. Pictured below is an example of this deformity before and after cosmetic surgery. This resulted from the collapse of the nasal septum caused by cartilage
inflammation. This patient has Granulomatosis with Polyangiitis, but an identical lesion may occur in Relapsing Polychondritis.
• Sinuses
• Chronic sinus inflammation that sometimes leads to a destructive process of tissues around the sinuses.
• Trachea
• A characteristic respiratory tract complication of Granulomatosis with Polyangiitis: narrowing of the “windpipe” just below the vocal cords, a condition called “subglottic
stenosis”. This narrowing, caused by inflammation and scarring, causes difficulty breathing and may, after a subacute progression, necessitate emergency tracheostomy.
Pictured below are two figures that show subglottic stenosis before (left) and after (right) surgery, performed by an Ear, Nose, & Throat specialist. The surgery
provided dramatic improvement in the patient’s breathing.
• Lungs
• A pneumonia–like syndrome, with lung “infiltrates“ can be seen on chest x–ray. Bleeding from the lungs can occur.
• Kidney
• Inflammation can occur in the kidney, leading to small (or rarely, large) amounts of blood and protein in the urine. This condition is called glomerulonephritis. If not
treated aggressively, Granulomatosis with Polyangiitis’s involvement of the kidneys can lead to kidney failure. Renal masses can occur, but are very unusual in this
disease.
• The image below is from a urinalysis of a patient with kidney inflammation. When Granulomatosis with Polyangiitis is active, red blood cells will form a clump or “cast”
(bracketed in white) within the tubules of inflamed kidneys. These “casts” pass through the renal system and may be viewed under the microscope in a patient’s urine.
• Skin
• Granulomatosis with Polyangiitis can cause many kinds of skin rashes. The most common rash occurs in the form of small purple or red dots on the lower extremities
(known as “palpable purpura”). Inadequate blood flow to fingers and toes can lead to Raynaud’s phenomenon (extreme sensitivity of the fingers to cold) and even
infarctions of the tips of fingers and toes, with the development of gangrene.
• with Polyangiitis can affect virtually any site in the body, but it has a
predisposition for certain organs. The classic organs involved in
Granulomatosis with Polyangiitis are the upper respiratory tract (sinuses, nose,
ears, and trachea [the “windpipe”]), the lungs, and the kidneys. Listed below
are the organs commonly involved in Granulomatosis with Polyangiitis and the
specific disease manifestation(s) in each organ.
• Ear
• Eye
• Nose
• Sinuses
• Trachea
• Lungs
• Kidney
• Skin
• Joints
• Nerves
• Miscellaneous
• Joints

• Arthritis can occur, with joint swelling and pain.

• Nerves

• Peripheral nerve involvement leads to numbness, tingling, shooting pains in the extremities, and sometimes to weakness in a foot, hand, arm, or leg.

• Miscellaneous

• Granulomatosis with Polyangiitis involvement of nearly all organs has been described, including the meninges (the layers of protective tissue around the brain and spinal cord), the prostate gland, and the genito–urinary tract. In
addition to involving specific organs, Granulomatosis with Polyangiitis also commonly results in generalized symptoms of fatigue, low–grade fever, and weight loss.

• The cause of Granulomatosis with Polyangiitis is not known. Compared to diseases with obvious genetic predispositions, genetics appear to play a relatively small role in the etiology of Granulomatosis with Polyangiitis . It is very
unusual for Granulomatosis with Polyangiitis to occur in two people in the same family. (It is possible, however, that less obvious genetic risk factors exist, e.g. genes that might pre-–dispose a patient to infection with an etiologic
organism). For some time, an infection has been suspected of causing (or at least contributing to) Granulomatosis with Polyangiitis , but no specific infection (bacterial, viral, fungal, or other) has been identified.

• How is Granulomatosis with Polyangiitis Diagnosed?

• Whenever possible, it is important to confirm the diagnosis of Granulomatosis with Polyangiitis by biopsying an involved organ and finding the pathologic features of this disease under the microscope. Because many diseases may
mimic Granulomatosis with Polyangiitis (and vice versa), before starting a treatment regimen it is essential to be as certain of the diagnosis as possible. We discuss some of the specific biopsy procedures used to diagnose
Granulomatosis with Polyangiitis in the section of this Websie entitled What is Vasculitis: Diagnosis?.

• Because Granulomatosis with Polyangiitis so often involves the upper respiratory tract (sinuses, nose, ears, and trachea [“windpipe”]) and because biopsy of these tissues is a relatively non–invasive procedure, these sites are
frequently biopsied in patients suspected of Granulomatosis with Polyangiitis . Unfortunately, the yield of biopsies from these sites is rather low: probably less than 50%. Therefore, sometimes more invasive procedures are required to
make the diagnosis.

• Lung biopsy (either open or thoracoscopic) is often the best way of diagnosing Granulomatosis with Polyangiitis . The ample amount of tissue obtainable through these procedures usually permits confirmation of the Granulomatosis
with Polyangiitis diagnosis. Similarly, although the amount of tissue obtained through a kidney biopsy is usually much smaller, the finding of certain pathologic features in the context of a patient’s overall symptoms, signs, and
laboratory tests is frequently diagnostic.

• Since 1982, when ANCAs (anti–neutrophil cytoplasmic antibodies) were first described, the role of these antibodies in the diagnosis of Granulomatosis with Polyangiitis has grown. ANCA testing, which involves the performance of a
simple blood test, has achieved wide availability during the 1990s. This is both good and bad: use of ANCA tests has led to earlier diagnoses and more rapid institution of appropriate treatment in many cases, but has also resulted in
misdiagnosis and incorrect treatment when the tests are not performed or interpreted correctly.

• As their name implies, ANCAs are directed against the cytoplasm (the non-nucleus part) of white blood cells. Their precise role in the disease process remains uncertain but is a topic of considerable research interest. ANCAs come in
two primary forms: 1) the C–ANCA [C stands for cytoplasmic] and, 2) the P–ANCA [P stands for perinuclear]. C–ANCAs have a particularly strong connection to Granulomatosis with Polyangiitis (up to 80% of patients — and possibly
more of those with active disease — have these antibodies). When C–ANCAs are present in the blood of a patient whose symptoms or signs suggest Granulomatosis with Polyangiitis , the likelihood of the diagnosis increases
considerably. In most cases, however, it is still VERY IMPORTANT to biopsy an involved organ to verify the diagnosis.

• Treatment and Course of Granulomatosis with Polyangiitis

• Until the 1970s, Granulomatosis with Polyangiitis was nearly always a fatal condition. The use of prednisone and other steroids helped prolong patients’ lives, but most patients eventually succumbed to the disease within a few months
or years. The first use of cyclophosphamide in the late 1960s began to change the terrible prognosis of this disease. Using the combination of cyclophosphamide and prednisone, more than 90% of patients with severe disease
respond to treatment, and 75% are able to achieve disease remissions. Unfortunately, Granulomatosis with Polyangiitis is a disease in which relapses frequently occur. Approximately half of all patients who achieve disease remissions
eventually suffer recurrences (“flares”). Flares of Granulomatosis with Polyangiitis are usually responsive to the same treatment that induced remission, but sometimes intensification of treatment (for example, changing to a more
powerful medication) is required.

• During the 1990s, physicians have increasingly used the combination of methotrexate and prednisone rather than cyclophosphamide and prednisone for Granulomatosis with Polyangiitis patients who do not have immediately life-
threatening disease (particularly disease that does not involve the kidneys severely), because of the frequency of severe side-effects associated with the latter regimen.

• Bactrim (or Septra), a combination of two antibiotics (trimethoprim and sulfamethoxazole) may also be helpful in the treatment of Granulomatosis with Polyangiitis , particularly in patients whose disease is limited primarily to the upper
respiratory tract. A large, multi-center study demonstrated that Bactrim is useful in preventing flares of Granulomatosis with Polyangiitis in the upper respiratory tract.

• What’s New in Granulomatosis with Polyangiitis?

• Pictured below is an example of this deformity before and
after cosmetic surgery. This resulted from the collapse of the
nasal septum caused by cartilage inflammation. This patient
has Granulomatosis with Polyangiitis, but an identical lesion
may occur in Relapsing Polychondritis.
• Ski
• Nerves
• Miscellaneous
• Ear
• Ear infections that are slow to resolve. Recurrent otitis media.
Decrease in hearing.
• Eye
• Inflammation can occur in different parts of the eye. Inflammation in the
white part of the eye is known as the sclera (“scleritis”). “Uveitis” is
inflammation within the eye. Inflammation behind the eye is known as
an “orbital pseudotumor”. An orbital pseudotumor such as those
caused by Granulomatosis with Polyangiitis can cause “proptosis”, or
protrusion of one eye.
• Skin
• Granulomatosis with Polyangiitis can cause many kinds of skin rashes. The most common
rash occurs in the form of small purple or red dots on the lower extremities (known as
“palpable purpura”). Inadequate blood flow to fingers and toes can lead to Raynaud’s
phenomenon (extreme sensitivity of the fingers to cold) and even infarctions of the tips of
fingers and toes, with the development of gangrene.
• Joints
• Arthritis can occur, with joint swelling and pain.
• Nerves
• Peripheral nerve involvement leads to numbness, tingling, shooting pains in the extremities,
and sometimes to weakness in a foot, hand, arm, or leg.
• Miscellaneous
• Granulomatosis with Polyangiitis involvement of nearly all organs has been described,
including the meninges (the layers of protective tissue around the brain and spinal cord), the
prostate gland, and the genito–urinary tract. In addition to involving specific organs,
Granulomatosis with Polyangiitis also commonly results in generalized symptoms of fatigue,
low–grade fever, and weight loss.
• The cause of Granulomatosis with Polyangiitis is not known. Compared to diseases with
obvious genetic predispositions, genetics appear to play a relatively small role in the etiology
of Granulomatosis with Polyangiitis . It is very unusual for Granulomatosis with Polyangiitis to
occur in two people in the same family. (It is possible, however, that less obvious genetic risk
factors exist, e.g. genes that might pre-–dispose a patient to infection with an etiologic
organism). For some time, an infection has been suspected of causing (or at least
contributing to) Granulomatosis with Polyangiitis , but no specific infection (bacterial, viral,
Recent advantages
• Nose
• Nasal crusting and frequent nosebleeds can occur. Erosion and perforation of the nasal septum. The bridge of the
nose can collapse resulting in a “saddle–nose deformity”. Pictured below is an example of this deformity before and
after cosmetic surgery. This resulted from the collapse of the nasal septum caused by cartilage inflammation. This
patient has Granulomatosis with Polyangiitis, but an identical lesion may occur in Relapsing Polychondritis.
• Sinuses
• Chronic sinus inflammation that sometimes leads to a destructive process of tissues around the sinuses.
• Trachea
• A characteristic respiratory tract complication of Granulomatosis with Polyangiitis: narrowing of the “windpipe” just
below the vocal cords, a condition called “subglottic stenosis”. This narrowing, caused by inflammation and scarring,
causes difficulty breathing and may, after a subacute progression, necessitate emergency tracheostomy. Pictured
below are two figures that show subglottic stenosis before (left) and after (right) surgery, performed by an Ear, Nose,
& Throat specialist. The surgery provided dramatic improvement in the patient’s breathing.
• Lungs
• A pneumonia–like syndrome, with lung “infiltrates“ can be seen on chest x–ray. Bleeding from the lungs can occur.
• Kidney
• Inflammation can occur in the kidney, leading to small (or rarely, large) amounts of blood and protein in the urine.
This condition is called glomerulonephritis. If not treated aggressively, Granulomatosis with Polyangiitis’s
involvement of the kidneys can lead to kidney failure. Renal masses can occur, but are very unusual in this disease.
• The image below is from a urinalysis of a patient with kidney inflammation. When Granulomatosis with Polyangiitis is
active, red blood cells will form a clump or “cast” (bracketed in white) within the tubules of inflamed kidneys. These
“casts” pass through the renal system and may be viewed under the microscope in a patient’s urine.
• How is Granulomatosis with Polyangiitis Diagnosed?
• Whenever possible, it is important to confirm the diagnosis of
Granulomatosis with Polyangiitis by biopsying an involved organ and
finding the pathologic features of this disease under the microscope.
Because many diseases may mimic Granulomatosis with Polyangiitis
(and vice versa), before starting a treatment regimen it is essential to be
as certain of the diagnosis as possible. We discuss some of the specific
biopsy procedures used to diagnose Granulomatosis with Polyangiitis in
the section of this Websie entitled What is Vasculitis: Diagnosis?.
• Because Granulomatosis with Polyangiitis so often involves the upper
respiratory tract (sinuses, nose, ears, and trachea [“windpipe”]) and
because biopsy of these tissues is a relatively non–invasive procedure,
these sites are frequently biopsied in patients suspected of
Granulomatosis with Polyangiitis . Unfortunately, the yield of biopsies
from these sites is rather low: probably less than 50%. Therefore,
sometimes more invasive procedures are required to make the diagnosis.
• Lung biopsy (either open or thoracoscopic) is often the best way of diagnosing Granulomatosis with Polyangiitis .
The ample amount of tissue obtainable through these procedures usually permits confirmation of the Granulomatosis
with Polyangiitis diagnosis. Similarly, although the amount of tissue obtained through a kidney biopsy is usually
much smaller, the finding of certain pathologic features in the context of a patient’s overall symptoms, signs, and
laboratory tests is frequently diagnostic.
• Since 1982, when ANCAs (anti–neutrophil cytoplasmic antibodies) were first described, the role of these antibodies in
the diagnosis of Granulomatosis with Polyangiitis has grown. ANCA testing, which involves the performance of a
simple blood test, has achieved wide availability during the 1990s. This is both good and bad: use of ANCA tests has
led to earlier diagnoses and more rapid institution of appropriate treatment in many cases, but has also resulted in
misdiagnosis and incorrect treatment when the tests are not performed or interpreted correctly.
• As their name implies, ANCAs are directed against the cytoplasm (the non-nucleus part) of white blood cells. Their
precise role in the disease process remains uncertain but is a topic of considerable research interest. ANCAs come in
two primary forms: 1) the C–ANCA [C stands for cytoplasmic] and, 2) the P–ANCA [P stands for perinuclear].
C–ANCAs have a particularly strong connection to Granulomatosis with Polyangiitis (up to 80% of patients — and
possibly more of those with active disease — have these antibodies). When C–ANCAs are present in the blood of a
patient whose symptoms or signs suggest Granulomatosis with Polyangiitis , the likelihood of the diagnosis increases
considerably. In most cases, however, it is still VERY IMPORTANT to biopsy an involved organ to verify the diagnosis.
• Treatment and Course of Granulomatosis with Polyangiitis
• Until the 1970s, Granulomatosis with Polyangiitis was nearly always a fatal condition. The use of
prednisone and other steroids helped prolong patients’ lives, but most patients eventually succumbed to
the disease within a few months or years. The first use of cyclophosphamide in the late 1960s began to
change the terrible prognosis of this disease. Using the combination of cyclophosphamide and
prednisone, more than 90% of patients with severe disease respond to treatment, and 75% are able to
achieve disease remissions. Unfortunately, Granulomatosis with Polyangiitis is a disease in which
relapses frequently occur. Approximately half of all patients who achieve disease remissions eventually
suffer recurrences (“flares”). Flares of Granulomatosis with Polyangiitis are usually responsive to the
same treatment that induced remission, but sometimes intensification of treatment (for example,
changing to a more powerful medication) is required.
• During the 1990s, physicians have increasingly used the combination of methotrexate and prednisone
rather than cyclophosphamide and prednisone for Granulomatosis with Polyangiitis patients who do not
have immediately life-threatening disease (particularly disease that does not involve the kidneys
severely), because of the frequency of severe side-effects associated with the latter regimen.
• Bactrim (or Septra), a combination of two antibiotics (trimethoprim and sulfamethoxazole) may also be
helpful in the treatment of Granulomatosis with Polyangiitis , particularly in patients whose disease is
limited primarily to the upper respiratory tract. A large, multi-center study demonstrated that Bactrim is
useful in preventing flares of Granulomatosis with Polyangiitis in the upper respiratory tract.
• What’s New in Granulomatosis with Polyangiitis?
• In the past few years, significant advances have been made in understanding Granulomatosis with
Polyangiitis , although many important questions remain. In addition to an improved understanding of
how to use the currently available medicines for Granulomatosis with Polyangiitis , it is likely that the
next few years will witness the development of new medicines for this disease. Scientific breakthroughs
may lead to the design of more specific modulators of the immune system that are of great benefit to
patients with Granulomatosis with Polyangiitis
• Risk factors
• Granulomatosis with polyangiitis can occur at any age. It most often
affects people between the ages of 40 and 65.
• Diagnosis
• Your doctor will ask you about your signs and symptoms, conduct a
physical exam, and take your medical history.
• Lab tests
• Blood tests can check for:
• Signs of inflammation, such as a high level of C-reactive protein or a high
erythrocyte sedimentation rate — commonly referred to as a sed rate.
• Anti-neutrophil cytoplasmic antibodies, which appear in the blood of most
people who have active granulomatosis with polyangiitis.
• Anemia, which is common in people with this disease.
• Signs that your kidneys aren’t properly filtering waste products from your
blood
• What are the features of granulomatosis with polyangiitis (GPA)?
• GPA primarily affects the upper respiratory tract (sinuses, nose, trachea
[upper air tube]), lungs, and kidneys. Any other organ in the body can be
affected as well.
• Granulomatosis with polyangiitis is an uncommon disorder that causes
inflammation of the blood vessels in your nose, sinuses, throat, lungs and
kidneys.
• Formerly called Wegener's granulomatosis, this condition is one of a
group of blood vessel disorders called vasculitis. It slows blood flow to
some of your organs. The affected tissues can develop areas of
inflammation called granulomas, which can affect how these organs work.
• Symptoms
• Signs and symptoms of granulomatosis with polyangiitis can develop suddenly or over several
months. The first warning signs usually involve your sinuses, throat or lungs. The condition often
worsens rapidly, affecting blood vessels and the organs they supply, such as the kidneys.
• Signs and symptoms of granulomatosis with polyangiitis might include:
• Pus-like drainage with crusts from your nose, stuffiness, sinus infections and nosebleeds
• Coughing, sometimes with bloody phlegm
• Shortness of breath or wheezing
• Fever
• Fatigue
• Joint pain
• Numbness in your limbs, fingers or toes
• Weight loss
• Blood in your urine
• Skin sores, bruising or rashes
• Eye redness, burning or pain, and vision problems
• Ear inflammation and hearing problems
• For some people, the disease affects only the lungs. When the kidneys are affected, blood and
urine tests can detect the problem. Without treatment, kidney or lung failure can occur.
• Granulomatosis with Polyangiitis (GPA, formerly called Wegener’s)
• Granulomatosis with polyangiitis (formerly called Wegener’s) is a rare disease
of uncertain cause that can affect people of all ages. It is characterized by
inflammation in various tissues, including blood vessels (vasculitis), but
primarily parts of the respiratory tract and the kidneys.
• Treatment
• With early diagnosis and appropriate treatment, you might recover from granulomatosis with polyangiitis
within a few months. Treatment might involve taking prescription drugs long term to prevent relapse.
Even if you're able to stop treatment, you'll need to regularly see your doctor — and possibly several
doctors, depending on which organs are affected — to monitor your condition.
• Medications
• Corticosteroids such as prednisone help suppress the immune system and reduce inflammation of the
blood vessels. Common side effects include weight gain, risk of infection and osteoporosis.
• Other drugs that suppress your immune system include cyclophosphamide, azathioprine (Azasan,
Imuran), mycophenolate (CellCept) and methotrexate (Trexall). Rituximab (Rituxan) is another option for
treating granulomatosis with polyangiitis. It's given by injection, and often is combined with
corticosteroids.
• Once your condition is controlled, you might remain on some drugs long term to prevent relapse. These
include rituximab, methotrexate, azathioprine and mycophenolate.
• Side effects of immune-suppressing drugs include increased risk of infection. Cyclophosphamide can
cause nausea, diarrhea and hair loss. Your doctor may prescribe other drugs to help prevent side
effects from prescribed treatments.
• Plasma exchange
• Also known as plasmapheresis, this treatment removes the liquid portion of your blood (plasma) that
contains disease-producing substances. You receive fresh plasma or a protein made by the liver
(albumin), which allows your body to produce new plasma. In people who have very serious
granulomatosis with polyangiitis, plasmapheresis can help the kidneys recover.