Vasculitis is an inflammation and necrosis of the
blood vessels, leading to hemorrhage, ischemia, and infarction..
Treatment of the condition is entirely dependent on the extent of the
disease. In many instances, cutaneous vasculitis is a self-limited
condition, relieved by leg elevation, avoidance of standing, and therapy
with non-steroidal anti-inflammatory drugs (NSAIDs). However, more
extensive or severe disease can prove more difficult to manage.
Treatment of vasculitis should begin with a confirmatory diagnosis to
eliminate conditions with a similar appearance. A tissue biopsy may
confirm diagnosis of cutaneous vasculitis, and can sometimes be used to
identify the type of immunoglobin involved. Once a diagnosis is
confirmed histologically, other organ systems should be evaluated to
identify potential causative factors.
The first and preferred treatment for cutaneous vasculitis should
always be avoidance of triggers known to exacerbate the condition, such
as excessive standing, infection, or drugs. For mild recurrent or
persistent disease, colchicine and dapsone are first-choice agents.
Severe cutaneous disease requires treatment with systemic
corticosteroids or more potent immunosuppression (azathioprine,
methotrexate, or cyclophosphamide are typical treatments). A combination
of corticosteroids and cyclophosphamide is required therapy for
systemic vasculitis, which is associated with a high risk of permanent
organ damage or death. Intravenous immunoglobin or plasmapheresis may be
useful in the treatment of severe, refractory vasculitis, or in
patients who have contraindications to traditional immunosuppression.
New biologic therapies that act via cytokine blockade or lymphocyte
depletion, such as the tumor necrosis factor-α inhibitor infliximab and
the anti-B-cell antibody rituximab, respectively, appear to offer some
benefit in certain settings, such as connective tissue disease and
anti-neutrophil cytoplasmic antibody-associated vasculitis.
This is article 3 in a series of four articles pertaining to vasculitis. To read the additional articles click title link, or click here.

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Vasculitis is the inflammation
of blood vessels. There are many types of vasculitis. Diseases
in which vasculitis is a primary process are called primary
systemic vasculitides. Vasculitis may also occur as a secondary
feature in other rheumatic diseases and syndromes | | | |
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What
is vasculitis?
Vasculitis
is a general term that refers to the inflammation of blood
vessels. When blood vessels become inflamed, they can only react
in limited ways. They may become weakened, stretch and increase
in size, or become narrow – even to the point of closing off
entirely.

What
are the consequences of vasculitis?
In an extreme situation, when a segment of a blood vessel
becomes weakened, it may then stretch and bulge (called an
“aneurysm”). The wall of the blood vessel can become so weak
that it ruptures and bleeds. Fortunately, this is a very rare
event.
If a blood
vessel becomes inflamed and narrowed, blood supply to that area
may be partially or completely eliminated. If collateral blood
vessels (thought of as alternate routes of blood supply) are not
available in sufficient quantity to carry the blood to such
sites, the tissue supplied by the affected blood vessels will
die. This is called infarction.

Because
vasculitis can occur in any part of the body, any tissue or
organ can be at risk.
Who
is affected by vasculitis?
Vasculitis can affect people of all ages from childhood to
adulthood. There are some types of vasculitis that occur in
certain age groups more than others.
What
are the causes of vasculitis?
Vasculitis may occur secondary to an identified underlying
disease or trigger. Occasionally, an allergic reaction to a
medicine may trigger vasculitis. Vasculitis can sometimes
develop in conjunction with an infection. Usually in these
cases, the infection causes an abnormal response in the
person’s immune system, damaging the blood vessels. Viral
hepatitis (a type of liver infection), is a specific infection
that can be associated with vasculitis. Vasculitis may also be
related to other diseases of the immune system that the patient
had for months or years. For example, vasculitis could be a
complication of rheumatoid arthritis, systemic lupus
erythematosus, or Sjögren’s syndrome.
In many
cases though, the causes of vasculitis are not known. These
diseases are collectively sometimes referred to under the broad
heading of primary forms of vasculitis. In such settings, the
appearance and location of the vasculitis often behaves in a
distinct way allowing it to be diagnosed as a unique type of
vasculitis and is given a specific name.
What
are the types of primary vasculitis?
There are many types of primary vasculitis including
disease entities such as Wegener’s granulomatosis,
microscopic polyangiitis, Henoch-Schönlein purpura,
polyarteritis nodosa, Kawasaki disease, giant cell
arteritis, Takayasu’s arteritis, and Behçet’s
disease. Some are named after doctors (Wegener, Takayasu,
Kawasaki) who were among those to provide the best
original descriptions of the illness or are named based
on features seen on biopsies (giant cell arteritis,
angiitis, arteritis nodosa) of affected tissues or blood
vessels. Although most of these are systemic (or
generalized) vasculitides where the vasculitis may
affect many organ systems at the same time, they often
differ a great deal among each other. Some of the
primary systemic vasculitic diseases may be quite mild
and require little or even no treatment. Other forms may
be severe, affecting critical organs and, if left
untreated, may lead to death within days or months.
Some
forms of primary vasculitis may be restricted in their
location to certain organs (these are called isolated
forms of vasculitis). Examples include vasculitis that
only occurs either in the skin, eye, brain (isolated CNS
vasculitis) or certain internal organs.
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What
are the symptoms of vasculitis?
Because any organ system may be involved, an enormous number of
symptoms are possible. If the skin is involved, there may be a
rash. If nerves suffer loss of blood supply, there may initially
be an abnormal sensation followed by a loss of sensation.
Vasculitis in the brain may cause a stroke, or in the heart may
result in a heart attack. Kidney inflammation usually is not
associated with symptoms and is detected by the doctor by
examination of the urine. This is important to recognize as
inflammation in the kidneys can lead to kidney failure unless
promptly detected.
Sometimes
the symptoms are nonspecific. When inflammation is present in
the body, we tend to respond in ways that tell us that we are
not well, but those responses may not be unique to vasculitis at
all. For example, along with the symptoms mentioned previously,
a person with vasculitis may also have a fever or experience
loss of appetite, weight loss and loss of energy.
How
is vasculitis treated?
Treatment depends entirely upon the diagnosis, the organs that
are affected, and the severity of the vasculitis. When
vasculitis represents an allergic reaction, it may be “self
limiting,” or will go away on its own and not require
treatment. There are other instances also where minimal to no
treatment is required and the person can be closely observed.
In
instances where critical organs such as the lungs, brain or
kidneys are involved, the outlook is less positive and
aggressive and timely treatment is necessary. For most forms of
systemic vasculitis, treatment generally includes corticosteroid
medications (prednisone is the most commonly prescribed).
For
some forms of vasculitis, treatment must also include another
immunosuppressive medication used in combination with the
prednisone. Some of these medications are chemotherapy agents
like those used to treat cancer, but are given in doses
considerably lower than people with cancer may receive. The
goal of this type of chemotherapy is to suppress the abnormal
immune response that has led to blood vessel damage.
What
is the outlook for people with vasculitis?
The outlook for a person who has vasculitis will vary with the
type of vasculitis that is present, what organs are being
affected, how severe the vasculitis is, and how the person
responds to treatment. Knowing the type of vasculitis allows the
doctor to predict the likelihood of illness severity and
outcome.
Prior to
the time of available treatment, people with severe vasculitis
may have had anticipated survival of only weeks to months.
However, today with proper treatment, normal life spans are
possible. The success of therapy is related to prompt diagnosis,
aggressive treatment and careful follow-up to be sure that side
effects from medications do not develop.
Once
vasculitis is under control (often referred to as
“remission”), medications may be cautiously withdrawn, with
the hope that the patient will sustain a long remission,
independent of treatment. Because some forms of vasculitis can
recur (referred to as a “relapse”) after a period of
remission, it is very important for patients with vasculitis to
remain under the care of a knowledgeable physician. |