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Polyarteritis Nodosa

Polyarteritis nodosa (PAN) is a rare disease caused by inflammation of the blood vessel (“vasculitis”) that causes organ system injury. Nerves, intestinal tract, heart, and joints are the areas most commonly affected by PAN. PAN may also affect the kidney’s blood vessels, resulting in high blood pressure and kidney function damage. It is a systemic necrotizing inflammation of the blood vessels (vasculitis) that affects medium-sized muscle arteries, typically involving the arteries of the kidneys and other internal organs, which generally inhibits the drainage of the lungs. PAN affects only medium-sized blood arteries, which was first described by Kussmaul and Maier in 1866. A patient’s autopsy with fever, weight loss, abdominal pain, and polyneuropathy showed regions of focal inflammatory exudations that produced visible nodules across medium-sized artery vessels.

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What Is Vasculitis?

Vasculitis is a general term relating to blood vessel inflammation. The blood vessel may become compromised when inflamed and expand in length, which may lead to aneurysms, or become so thin that it splits into the tissue that it bleeds. Vasculitis can also cause the blood vessel to close so that the lungs are weakened by the lack of oxygen and blood-supplied nutrients. PAN affects only medium-sized blood vessels.

Polyarteritis Nodosa Symptoms

Because there may be several different organ systems involved, PAN can have a wide range of symptoms. Patients with PAN may feel sick and tired in general, have a fever, or lose appetite and weight. Some may have symptoms related to areas of involvement such as muscle and/or joint pain, skin sores that may be present as rough nodules or ulcers, abdominal pain or blood in stools which occur as a result of involvement in the intestinal tract, or shortness of breath or chest pain from heart disease. High blood pressure is common in PAN and is usually caused by reduced blood flow to the kidneys due to vasculitis.

PAN’s signs are very noticeable and may include:

  • a weakened appetite
  • abrupt loss of weight
  • abdominal pain
  • excessive fatigue
  • fever
  • muscle and joint aches

Symptoms of Polyarteritis Nodosa

PAN influences the nervous systems of nearly 70% of people, according to Johns Hopkins. PAN can induce seizures and cause neurological problems after two to three years if left untreated, causing reduced alertness and cognitive dysfunction. There are also very common skin lesions. PAN affects the skin on the legs more often, and the sores may be painful.

Causes of Polyarteritis Nodosa

There is no knowledge of the exact cause of polyarteritis nodosa. No predisposing factor was observed in most cases. Unidentified diseases of bacteria and/or viruses can be a cause. In drug abusers, particularly those who use amphetamines, and in patients with hepatitis B (liver infection), polyarteritis nodosa was observed. (For more information on this disease, pick “Hepatitis B” as the Rare Disease Database search term.) This condition was also associated with an allergic reaction to certain medications and vaccines. PAN’s cause is unknown. PAN is not a cancer form, it is not contagious, and it usually does not occur in families. Research laboratory evidence strongly supports the immune system playing a critical role in PAN, causing inflammation and damage to the blood vessel and tissue. PAN is strongly associated with infection with hepatitis B. Since the vaccine for hepatitis B was established, PAN rates have significantly decreased.

Diagnosis of Polyarteritis Nodosa

PAN is a complex condition requiring multiple studies before a precise diagnosis can be made by the physician. The doctor will probably prescribe a complete blood count to test the red blood cells and white blood cells.

You can also be asked to undergo a tissue biopsy that involves using a small sample of an afflicted artery for lab testing, an arteriogram – an artery X-ray, to measure inflammation – an erythrocyte sedimentation rate (ESR) test. Skin and muscle or nerve biopsies may be helpful when making a diagnosis, according to Johns Hopkins. Most of the people with PAN have high ESR outcomes. Skin and muscle or nerve biopsies can be effective during the diagnosis, according to Johns Hopkins. Once these tests are performed, a subsequent diagnosis and treatment plan will be formulated by your doctor. In a few cases, doctors may misinterpret gastrointestinal side effects and abdominal pain for inflammatory bowel disease. This is why it is important to report immediately to your doctor about any long-term gastrointestinal effects.

A doctor may suspect PAN based on information from a variety of sources, including medical history to look for symptoms of PAN.

  • Physical examination to detect sites involving organs and to exclude other diseases that may appear similar.
  • Blood tests and urinalysis to search for characteristics that may suggest inflammation or organ involvement sites.
  • Imaging tests such as X-rays, CT scans, or MR scans that indicate anomalies in affected areas.

Once the diagnosis of PAN is suspected, diagnosis confirmation is usually followed by an arteriogram or a biopsy. An arteriogram involves injecting a dye into the bloodstream that allows blood vessel visualization and may show vasculitis suggestive narrowing of aneurysms or the blood vessel. A biopsy of an affected area may be performed to try to confirm the presence of vasculitis in the tissue, based on the place of involvement. Biopsies and arteriograms are only indicated in organ locations where scans, laboratory tests or other forms of imaging studies report abnormal outcomes.

Treatment of Polyarteritis Nodosa

A mixture of prescription drugs, including corticosteroids immune suppressants antiviral drugs, is the most common treatment for PAN.

  • corticosteroids
  • immune suppressants
  • antiviral medications

through reducing inflammation and removing other receptors in the body, high doses of steroids or corticosteroids regulate the effects of PAN. Corticosteroids can generate a number of side effects, especially when taken orally. Corticosteroids may help keep the immune system from destroying your lungs, it may take certain kinds of immunosuppressive drugs. Patients with PAN with essential activity in the organ system are generally treated with a corticosteroid such as prednisone or prednisolone in conjunction with other immunosuppressive drugs such as cyclophosphamide (Cytoxan ®). Cyclophosphamide is given for certain types of cancer at high doses as a cure and is therefore often referred to as “chemotherapy.”

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