What Is Necrotizing Vasculitis?
vasculitis (SNV) is an inflammation of blood vessel walls. It
typically affects small and medium vessels. This inflammation can interrupt your
normal blood flow. It results in skin and muscle damage, including necrosis. Necrosis is the death of
tissues and organs. The inflammation can also cause the vessels to thicken and
scar. This can eventually cause your vessels to die over time.
The affected blood vessels may be located in any part of
your body. The effect of necrotizing vasculitis depends on where the affected
blood vessels are located and how much damage they cause.
This is a rare disease and there’s no known cause. However,
autoimmunity is considered to play a role in this disorder. Autoimmunity occurs when your
body forms antibodies and attacks your own tissues and organs.
What Causes Necrotizing Vasculitis?
Doctors don’t know what causes this condition.
You’re more likely to develop this disease if you have an
autoimmune condition, such as rheumatoid arthritis or systemic lupus
Other conditions associated with SNV include:
disease, or mucocutaneous lymph node syndrome
Necrotizing Vasculitis in Children
SNV is very rare in children, but it does occur. According
to a recent study, children
diagnosed with Kawasaki disease are more at risk for SNV. Kawasaki disease is
the leading cause of heart disease in children in the developed world.
What Are the Symptoms of Necrotizing Vasculitis?
Because this condition affects your blood vessels, symptoms
might occur in various parts of your body. There’s no single set of symptoms that
can definitely indicate you have necrotizing vasculitis.
You might notice initial symptoms on your own without a
medical test. These include:
Other early symptoms are only detectable through a blood
test. These include anemia and leukocytosis, which involves having a high
number of white blood cells.
As the disease progresses, symptoms can worsen and become
more varied. Your specific symptoms depend on what parts of your body affected.
You may have:
- skin discoloration
- lesions, which are usually seen on the legs
In some cases, the condition may be limited to your skin. In
other cases, you might develop kidney damage or bleeding in your lungs. If your
brain is affected, you may have difficulty swallowing, speaking, or moving.
How Is Necrotizing Vasculitis Diagnosed?
In most cases, your doctor will first take a sample of your
blood. This sample will be tested for antineutrophil cytoplasmic antibodies (ANCAs).
You may have this condition if these antibodies are found in your blood sample.
Your doctor may suspect you have this condition whenever
your ANCA test comes back positive and your symptoms affect at least two organs
or other parts of your body.
Your doctor might perform further tests to help confirm your
diagnosis. These tests might include a biopsy of the affected area or an X-ray. A hepatitis blood test and a urine analysis may also be
performed. In some cases, a chest X-ray will identify if there are underlying
blood flow issues happening in your body.
How Is Necrotizing Vasculitis Treated?
Once your diagnosis has been confirmed, your doctor will
decide whether your treatment goals are maintenance or remission. Maintenance
treatment aims to prevent further damage to your blood vessels. Remission treatment
aims to identify and prevent the reaction that’s causing the necrosis.
Necrotizing vasculitis is usually treated with a type of steroid
called a corticosteroid. This type of medication help reduce inflammation. Corticosteroids
are prescribed at high doses at first. If the disease becomes less severe, your
doctor can gradually decrease your medication dosage.
Your doctor may introduce other drugs that suppress the
immune system, such as antihistamines, if the steroids are ineffective.
You may also need to begin taking cyclophosphamide if your symptoms don’t
improve or if they’re very severe. This
is a chemotherapy drug used to treat cancers. Cyclophosphamide is proven
effective in treating certain forms of vasculitis. You’ll continue taking these
medications even after your symptoms go away. You should take them for at least
a year after you stop having symptoms.
Close monitoring for involvement of the following should be
- nervous system
If any conditions affecting these areas develop, your doctor
can prescribe appropriate treatment for those as well.
What Is the Outlook for Those with Necrotizing Vasculitis?
This rare condition is treatable. However, damage to any
area affected by SNV can’t be reversed.
The outlook for those with this diagnosis varies and depends
on the severity of tissue damage before their treatment begins.
Possible complications include infections due to having a suppressed
immune system during treatment and secondary infections of necrotized tissue.
A recent study also indicates that those with SNV are at a higher risk
for developing malignant growths and cancers.