Polyarteritis Nodosa
Medical Author: William C. Shiel Jr., MD, FACP, FACR
I have been diagnosed with polyarteritis nodosa (PAN). How does PAN differ from lupus?
Medical Author: William C. Shiel Jr., MD, FACP, FACR
Medical Editor: Jay W. Marks, MD
Polyarteritis nodosa is a type of inflammation of the blood vessels (vasculitis) that affects medium- and large-sized arteries. Depending on which blood vessels are involved and specifically the tissues they supply, injury can occur to internal organs, such as the kidneys or bowel.
What is polyarteritis nodosa?
Polyarteritis nodosa is a rare autoimmune disease (immune
system attacking its own body) featuring spontaneous
inflammation of the arteries (arteritis). Because arteries are
involved, the disease can affect any organ of the body. The most common areas
of involvement include the muscles, joints, intestines (bowels),
nerves, kidneys, and skin. Poor function or pain in any of these organs can
be a symptom. Poor blood supply to the bowels can cause abdominal pain, local bowel death, and bleeding. Fatigue, weight loss, and fever are common.
Polyarteritis nodosa is most common in
middle-age people. Its cause is unknown, but it has been reported after
hepatitis B infection. Polyarteritis is not felt to be an inherited condition.
I have been diagnosed with polyarteritis nodosa (PAN). How does PAN differ from lupus?
Medical Author: William C. Shiel Jr., MD, FACP, FACR
Medical Editor: Jay W. Marks, MD
Polyarteritis nodosa is a type of inflammation of the blood vessels (vasculitis) that affects medium- and large-sized arteries. Depending on which blood vessels are involved and specifically the tissues they supply, injury can occur to internal organs, such as the kidneys or bowel.
How is polyarteritis nodosa diagnosed?
The diagnosis is supported by tests that indicate inflammation
including elevation of blood sedimentation rate and
C-reactive protein. The white blood cell count and platelet count can be elevated, while the
red blood count is decreased (anemia). Hepatitis B virus testing (for either antigen and antibody) can be found in 10%-20% of patients with polyarteritis nodosa. Urine testing can
show protein and red blood cells in the urine. In patients with
nerves affected, nerve function tests are abnormal.
The diagnosis of polyarteritis nodosa is confirmed by a biopsy of involved
tissue that reveals the inflamed blood vessels (vasculitis). Examples of tissues that are sometimes biopsied include nerves, muscle, kidneys, and bowel. Vasculitis
of the bowel and kidneys can often be detected with an angiogram (x-ray testing while contrast "dye" is infused into the blood
vessels).
The American College of Rheumatology established criteria for
the classification of polyarteritis nodosa in 1990. For
classification purposes, a patient is said to have polyarteritis nodosa if at
least three of the following 10 criteria are present:
- Weight loss greater than/equal to 4 kg
- Livedo reticularis (a mottled purplish skin discoloration over the
extremities or torso)
- Testicular pain or tenderness (occasionally, a site
biopsied for diagnosis)
- Muscle pain, weakness,
or leg tenderness
- Nerve disease (either single or multiple)
- Diastolic blood pressure greater than 90mm Hg (high blood pressure)
- Elevated kidney blood tests (BUN greater than 40 mg/dl or creatinine
greater than 1.5 mg/dl)
- Hepatitis B virus tests positive (for surface antigen or antibody)
- Arteriogram (angiogram) showing the arteries that are dilated (aneurysms)
or constricted by the blood vessel inflammation
- Biopsy of tissue showing the arteritis (typically inflamed arteries)
Next: How is polyarteritis treated? »
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Polyarteritis Nodosa
Vasculitis »
What is vasculitis?
Vasculitis is a general term for a group of uncommon diseases that
feature inflammation of the
blood vessels. The blood vessels of the
body are referred to as the vascular system. The
blood vessels are comprised of arteries that pass oxygen-rich blood to the
tissues of the body and veins that return oxygen-depleted blood from the
tissues to the lungs for oxygen. Vasculitis is characterized by
inflammation in and damage to the walls of various blood vessels.
Each of the vasculitis diseases is defined by certain patterns of distribution of
blood vessel involvement, particular organ involvement, and laboratory test abnormalities.
As a group, these diseases are referred to as vasculitides. The word vasculitis is derived from the Latin
"vasculum", vessel + "- itis", inflammation. Another term for vasculitis is
angiitis. When arteries are the inflamed blood vessels, the condition is also referred to as arteritis. When the veins are ...
Read the Vasculitis article »
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