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Polyarteritis nodosa is an autoimmune disease that affects arteries.
- Common areas affected include the muscles, joints, intestines (bowels), nerves, kidneys, and skin.
- Diagnosis of polyarteritis nodosa is confirmed by a biopsy of involved tissue or angiography.
- Treatment is directed toward decreasing the inflammation of the arteries.
PAN is characterized by widespread AND spontaneous inflammation, weakening, and degeneration of small- and medium-sized arteries(arteritis).
Blood vessels in any organ or organ system may be affected including arteries supplying the kidneys, heart, intestine, nervous system, and/or skeletal muscles. Common areas of involvement muscle joints intestines (bowels) nerves kidneys skin.
Common Signs and Symptoms
Poor function or pain in any of the organs mentioned above can be a symptom. Fatigue, weight loss, and fever a general feeling of ill health (malaise), headache muscle aches (myalgias) and/or abdominal pain.Damage to affected arteries may result in abnormally increased blood pressure (hypertension), "ballooning" (aneurysm) of an arterial wall, the formation of blood clots (thrombosis), obstruction of blood supply to certain tissues, and/or tissue damage and loss (necrosis) in certain affected areas.
Other symptoms and findings are often present, depending upon which areas of the body are affected.
How is polyarteritis nodosa diagnosed?
Tests showing inflammation elevation of blood sedimentation rate and c-reactive protein. white blood cell count and platelet count can be elevated, the red blood count is decreased (anemia). The Hepatitis B virus tests (antigen and antibody) can be found in 10-20% of patients. 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 which reveals the inflamed blood vessels (vasculitis). The 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 exact cause of polyarteritis is not known. There is a possibility of bacterial or viral infection as being the cause. Allergic reactions and vaccines have been linked to the disorder. Autoimmune diseases related to polyarteritis are Wegener's granulomatosis, Churg-Strauss syndrome, Takayasu's arteritis, and temporal or giant cell arteritis.
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 3 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 90mmHg (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).
How is polyarteritis treated?
Polyarteritis is a serious illness that can be fatal. Treatment is directed toward decreasing the inflammation of the arteries by suppressing the immune system. Medications used to treat polyarteritis nodosa include high-dose intravenous and oral cortisone medications, such as prednisone and immunosuppressive drugs, such as cyclophosphamide (CYTOXAN).
Source references:
National Organization for Rare Disorders
American Autoimmune Related Disease Association
MedicineNet
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Polyarteritis nodosa Resources:
“Polyarteritis nodosa ”
by Dr David Gotlieb MBChB FCP(SA),
Rheumatologist & Specialist Internal Medicine
A description of the illness and treatments by a Rheumatologist in Cape Town, South Africa who calls himself drdoc on-line.
