Polyarteritis Nodosa (PAN)
Other Names: Periarteritis Nodosa, PAN
Polyarteritis Nodosa (PAN) is a form of vasculitis
which is an autoimmune that attacks the medium-sized
arteries. It is a serious disorder of the blood vessels
that cause the vessels to become swollen and damaged,
weakening the walls of the vessels themselves. This
can lead to serious complications such as strokes,
kidney failure, heart attacks, intestinal necrosis,
pericarditis, aneurysm and others. Left untreated
can be fatal.
PAN is more common in adults than children, and the
initial symptoms might include fever, fatigue, weakness,
loss of appetite, unintentional weight loss and skin
rashes. Abdominal discomfort may also be present with
or without eating.
PAN can also have nervous system involvement which
makes diagnosing the disorder more difficult (can
mimic one of the many CNS autoimmune disorders or
other CNS disorders). When there is nerve involvement,
symptoms might include numbness, pain, burning and
weakness. It could involve the whole body or just
one side depending on which vessels are involved.
There are many forms of Vasculitis, I encourage you
to visit the Vasculitis page for information about
other forms of Vasculitis autoimmune's as it is not
uncommon to have more than one form of Vasculitis
at the same time. Vasculitis can also present with
other autoimmune conditions as well.
Symptoms:
Abdominal pain
Fatigue
Fever
General body pain
General joint pain and stiffness
General numbness and weakness
Loss of appetite
Nodules under the skin
Rashes on the arms
Rashes on the face
Rashes on the legs
Unintentional Weight loss
Diagnosis:
Diagnosing PAN can be difficult and lengthy since
many of the symptoms and test results mimic other
disorders. Most of the diagnosis will depend on symptom
history and contrast imaging of vessels themselves
to view any weakness and flow patterns. Tissue biopsies
may also be taken to help eliminate other disorders
and confirm a PAN diagnosis.
According to the 1990
American College of Rheumatology criteria *, a
PAN diagnosis should meet the following criteria:
1. Weight loss > 4 kg: Loss of >4 kg body
weight since illness began, not related to dieting
or other factors.
2. Livedo reticularis: Mottled reticular pattern
over the skin of portions of the extremities or torso.
3. Testicular pain/tenderness: Pain or tenderness
of the testicles, not due to infection, trauma or
other causes.
4. Myalgias, weakness or leg tenderness: Diffuse
myalgias (excluding shoulder or hip girdle) or weakness
of muscles or tenderness of leg muscles.
5. Mono- or polyneuropathy: Development of mononeuropathy,
multiple mononeuropathies or polyneuropathy.
6. Diastolic BP >90 mmHg: Development of hypertension
with the diastolic BP higher than 90 mmHg.
7. Elevated BUN or creatinine: Elevation of BUN >40
mg/dl or creatinine >1.5 mg/dl, not due to dehydration
or obstruction.
8. Hepatitis B virus: Presence of hepatitis B surface
antigen or antibody in serum.
9. Arteriographic abnormality: Arteriogram showing
aneurysms or occlusions of the visceral arteries,
not due to arteriosclerosis, fibromuscular dysplasia
or other non-inflammatory causes.
10. Biopsy of small or medium-sized artery containing
polymorphonuclear cells: Histologic changes showing
the presence of granulocytes or granulocytes and mononuclear
leucocytes in the artery wall.
(Quoted directly from the ACR website, credit given
as a link above)
Blood tests may also show:
Elevated Erythrocyte Sedimentation Rate (ESR)
Elevated White Blood Cell Count (WC)
Elevated C-Reactive Protein (CRP)
Treatment:
Treating PAN requires the use of immunosuppressant
therapy to reduce the body's immune response that
is causing the attack against the vessels. NSAIDS
or Prednisone may also be used to reduce the inflammation
to prevent vessels from rupturing. Other treatments
involve symptom management for the GI problems or
nerve problems that result from PAN
Prognosis:
With treatment, PAN is usually a controllable disease
and the prognosis can be quite good with a good chance
of long-terms survival. The more serious cases where
kidneys and the heart are already involved may not
be as well. Without treatment, the prognosis is not
good at all and can be fatal
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