Signs of Wegener's granulomatosis
Symptoms of Wegener's granulomatosis
The list of medical symptoms mentioned in various sources
for Wegener's granulomatosis may include:
List of 102
Wegener's granulomatosis Symptoms
Note that Wegener's granulomatosis symptoms usually refers to various medical symptoms known to a patient,
but the phrase Wegener's granulomatosis signs may often refer to those signs
that are only noticable by a doctor.
Signs or Symptoms of Wegener's granulomatosis:
The initial symptoms of Wegener's granulomatosis are often
vague or nonspecific and frequently include upper respiratory
tract symptoms, joint pains, weakness, and fatigue.
Upper respiratory tract. The most common sign of
Wegener’s granulomatosis is involvement of the upper respiratory
tract, which occurs in nearly all patients. Symptoms include sinus
pain, discolored or bloody nasal drainage and, occasionally, nasal
ulcerations. A common manifestation of the disease is persistent
rhinorrhea ("runny nose") or other cold symptoms that do not
respond to standard treatment or that become progressively worse.
Rhinorrhea can result from nasal inflammation or sinus drainage
and can cause pain. A hole or perforation of the nasal septum may
develop, and collapse of the nasal bridge (called saddlenose
deformity) may occur in some individuals. Blockage of the
eustachian tubes, which are important for normal ear function, may
cause chronic ear problems and hearing loss. A secondary bacterial
infection can cause Wegener’s-related sinusitis (inflammation of
the sinuses) with congestion and chronic sinus pain.
Lungs.The lungs are affected in most patients with
Wegener’s granulomatosis, although no symptoms may be present. If
symptoms are present, they include cough, hemoptysis (coughing up
of blood), shortness of breath, and chest discomfort.
Kidneys.Kidney involvement, which occurs in more than
three-fourths of patients, usually does not cause symptoms. If
detected by blood tests, proper treatment can be started,
preventing long-term damage to the kidneys.
Musculoskeletal system.Pain in the muscles and joints
or, occasionally, joint swelling affects two-thirds of patients
with Wegener’s granulomatosis. Although joint pain can be very
uncomfortable, it does not lead to permanent joint damage or
deformities.
Eyes.Wegener’s granulomatosis can affect the eyes in
several ways. Patients may develop conjunctivitis (inflammation of
the conjunctiva, the inner lining of the eyelid), scleritis
(inflammation of the scleral layer, the white part of the
eyeball), episcleritis (inflammation of the episcleral layer, the
outer surface of the sclera), or as an orbital mass lesion (sore
behind the eye globe). The symptoms of eye involvement include
redness, burning or pain in the eye. Double vision or a decrease
in vision are serious symptoms requiring immediate medical
attention.
Skin lesions.Nearly half of people with Wegener’s
granulomatosis develop skin lesions. These small red or purple
raised areas or blister-like lesions, ulcers, or nodules may or
may not be painful.
Other symptoms.Some patients
experience narrowing of the trachea (subglottic stenosis).
The symptoms can include voice change, hoarseness, shortness of
breath, or cough.
The nervous system and heart occasionally may be
affected. Fever and night sweats also may occur. However,
fever also may signal an underlying infection, often of the upper
respiratory tract.
Diagnosis
To treat people with Wegener’s granulomatosis most effectively,
doctors must diagnose the disease early in its course. There are
no blood tests that a doctor can use to diagnose Wegener’s
granulomatosis, but blood tests are important to rule out other
causes of illness and to determine which organ sites may be
affected. Most blood tests are nonspecific and can only suggest
that a person has an inflammatory process. Anemia (low red blood
cell count), elevated white blood cell count and platelet count,
and an elevated sedimentation rate are commonly found in people
with Wegener’s granulomatosis. If the kidneys are involved, red
blood cells and structures called red blood cell casts are visible
in the urine when viewed under a microscope, and the blood tests
measuring kidney function (creatinine and BUN) may show
abnormalities.
X-ray results can be very helpful in diagnosing Wegener’s
granulomatosis. People with lung involvement will have abnormal
chest x-rays, which may show one or many fluffy infiltrates, solid
nodules, or cavities. Sinus x-rays or computed tomography (CT)
scans in people with sinus involvement may show thickening of the
sinus lining.
Many patients with active Wegener's granulomatosis have a blood
test that reveals the presence of a specific type of antibody
called antineutrophil cytoplasmic antibodies (ANCA) (an antibody
is a disease-fighting protein). Although a positive ANCA test is
useful in supporting a suspected diagnosis of Wegener’s
granulomatosis, in most instances it is not used by itself to make
a diagnosis of this disorder. The ANCA test may be negative in
some patients with active Wegener’s granulomatosis.
Currently, the only definite way to diagnose Wegener’s
granulomatosis is by performing a biopsy of an involved organ site
(usually the sinuses, lung, or kidney). The tissue is examined
under the microscope to confirm the presence of vasculitis and
granulomas (a specific type of inflammation), which together are
diagnostic features of the disease. A biopsy is very important
both to confirm the presence of Wegener’s granulomatosis and also
to assure the absence of other disorders that may have similar
signs and symptoms.
Treatment
With the appropriate treatment, the outlook is good for
patients with Wegener’s granulomatosis. In a study of 158 patients
who were treated at the National Institutes of Health (NIH), 91
percent of them markedly improved. After 6 months to 24 years of
follow-up, 80 percent of the patients survived.
In most cases, standard therapy consists of a combination of a
glucocorticoid drug that reduces inflammation and a cytotoxic drug
that interferes with the abnormal growth of cells.
Prednisone is the most common glucocorticoid drug (a steroid)
that is used. Prednisone is similar to hydrocortisone, the natural
glucocorticoid hormone produced by the body. It is chemically
different from the anabolic steroids that have been used by
athletes and is given in doses much higher than the body normally
produces. Prednisone is usually administered as a single morning
dose in an attempt to imitate how the body normally secretes
hydrocortisone. When the person’s illness improves, the prednisone
dose is gradually decreased and converted to an every other day
dosing schedule, usually over a period of 3 to 4 months. With
further improvement in the disease, the prednisone is very
gradually decreased and discontinued completely after
approximately 6 to 12 months. When prednisone is taken by mouth,
the body stops making its own natural hydrocortisone. As the
prednisone dose is gradually reduced the body will resume making
hydrocortisone again. It is extremely important that prednisone
never be stopped suddenly because the body requires prednisone (or
hydrocortisone) for its function and may not be able to
immediately make what it needs.
Cyclophosphamide (CytoxanÔ) is the most commonly used cytotoxic
drug. Cyclophosphamide is taken once a day by mouth. It is
important for a patient to take the drug all at once in the
morning followed by drinking a large amount of fluid. Although the
initial dose of cyclophosphamide is based on the patient’s weight
and kidney function, the doctor may adjust the dosage based on the
blood counts, which are monitored closely to be sure that the
white blood cell count is maintained at a safe level.
Cyclophosphamide is continued for a full year beyond that point at
which the disease has become quiet (is in remission). The dose of
cyclophosphamide is then decreased gradually and eventually
discontinued.
Cyclophosphamide and prednisone are both powerful drugs that
suppress the immune system. Although these medications are
beneficial in treating Wegener’s granulomatosis, patients and
their doctors should be aware that the drugs potentially have
serious side effects. Careful monitoring by the doctor is very
important. Because these drugs suppress the immune system, they
can affect the body’s ability to fight off infection. Patients
should report immediately any symptoms of infection and,
specifically, any fever to their doctors. Prednisone can cause
weight gain, cataracts, brittle bones, diabetes, and alterations
in mood and personality. Cyclophosphamide can cause bone marrow
suppression (lowering of blood counts), sterility, hemorrhagic
cystitis (bleeding from the bladder) as well as other serious side
effects.
Approximately half of people with Wegener’s granulomatosis may
experience a return (relapse) of their disease. This occurs most
frequently within two years of stopping medication, but
potentially can occur at any point both during treatment or after
stopping treatment. Thus, it is extremely important that patients
continue to see their physicians regularly, both while they are on
these medications, as well as after the medications have been
stopped. Even while on medication, many patients are able to lead
relatively normal lives and will remain in remission after therapy
has been stopped completely.
Research
Since the 1970s, research physicians at the National Institute
of Allergy and Infectious Diseases (NIAID), a component of NIH,
have been interested in Wegener’s granulomatosis. NIAID scientists
first introduced the combination of a glucocorticoid with
cyclophosphamide for treating people with this disease. This
dramatic breakthrough remains the standard of treatment. Despite
this, researchers realize that these medicines have serious side
effects and cannot be tolerated by all people. Therefore, NIH
researchers continue to study Wegener’s granulomatosis to
understand the causes of the disease and to develop new
treatments. NIAID is conducting several studies to investigate new
treatment regimens. These studies each have individual entry
criteria but are open to patients who have a definitive diagnosis
of Wegener’s granulomatosis and who have active disease.
Further Information
For information about enrolling in an NIAID clinical study at
NIH's facility in Bethesda, Maryland, the patient's personal
physician should call or write to:
Dr. Michael C. Sneller
Head, Immunologic Diseases
Section
or
Dr. Carol A. Langford
Immunologic Diseases
Section
Laboratory of Immunoregulation
National Institute of
Allergy and Infectious Diseases
National Institutes of
Health
Building 10, Room 11B-13, MSC 1876
10 Center
Drive
Bethesda, MD 20892-1876
301/496-1124
For more information on patient support groups, contact:
Wegener's Foundation, Inc.
Attention: Ms. Linda
Baltrusch
3705 South George Mason Drive
Suite 1813
South
Falls Church, VA 22041
703/931-5852
Wegener's Granolomatosis Support Group, Inc.
(International)
P.O. Box 28660
Kansas City, MO
64188-8660
800/277-WGSG (9474)
http://www.wgsg.org/
(Source: excerpt from
Wegener's Granulomatosis, NIAID Fact Sheet: NIAID)
More Symptoms of Wegener's granulomatosis:
More detailed symptom information may be found on the symptoms of Wegener's granulomatosis article.
In addition to the above medical information, to get a full picture
of the possible signs or symptoms of this condition
and also possibly the signs and symptoms of its related medical conditions,
it may be necessary to examine symptoms
that may be
caused by:
Medical articles on signs and symptoms:
These general reference articles may be related
to medical signs and symptoms of disease in general:
What are the signs of Wegener's granulomatosis?
The phrase "signs of Wegener's granulomatosis" should, strictly speaking,
refer only to those signs and symptoms of Wegener's granulomatosis that are not readily apparent to the patient.
The word "symptoms of Wegener's granulomatosis" is the more general meaning; see symptoms of Wegener's granulomatosis.
The signs and symptom information on this page
attempts to provide a list of some possible signs and symptoms of Wegener's granulomatosis.
This medical information about signs and symptoms for Wegener's granulomatosis has been gathered
from various sources,
may not be fully accurate,
and may not be the full list of Wegener's granulomatosis signs or Wegener's granulomatosis symptoms.
Furthermore, signs and symptoms of Wegener's granulomatosis may vary on an individual basis for each patient.
Only your doctor can provide adequate diagnosis of any signs or symptoms and whether they
are indeed Wegener's granulomatosis symptoms.
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