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Clinical Services at Rush Types of Vasculitis

Polyarteritis nodosa (PAN)

What is the cause of polyarteritis nodosa?
What are the symptoms of polyarteritis nodosa?
How is the diagnosis of polyarteritis nodosa made?
What is the treatment for polyarteritis nodosa?

What is the prognosis for polyarteritis nodosa?
 

Polyarteritis nodosa (panarteritis nodosa; periarteritis nodosa) is a disease in which arteries become swollen and damaged from attack by rogue immune cells. Constricted arteries can lead to damage of the organs they serve, often the skin, heart, kidneys and nervous system.

About a third of people with the disease have been diagnosed with active hepatitis B. Men are twice as likely as women to develop polyarteritis nodosa, and the onset of the disease usually occurs between the ages of 30 and 49.

What is the cause of polyarteritis nodosa?

The cause of Polyarteritis nodosa is unknown. It is an autoimmune disorder, where certain immune cells attack blood vessels throughout the body. It is not known what triggers this reaction. Some cases of polyarteritis nodosa may be associated with hepatitis B and less frequently with hepatitis C. It is possible that the hepatitis virus itself or the immune response plays an important role in causing the disease.

What are the symptoms of polyarteritis nodosa?

Generalized symptoms include:

  • Rashes
  • Skin swelling, ulcers and lumps
  • Abdominal pain
  • Decreased appetite
  • Fatigue
  • Fever
  • Joint aches
  • Muscle aches
  • Unintentional weight loss (8 lbs. or more)
  • Weakness

Nerve involvement may cause sensory changes with numbness, pain, burning and weakness. Central nervous system involvement may cause strokes or seizures. Kidney involvement can produce varying degrees of renal (kidney) failure.

When heart arteries are involved, heart attack, heart failure and inflammation of the sac around the heart (pericarditis) can occur.

How is the diagnosis of polyarteritis nodosa made?

There are no specific lab tests for diagnosing polyarteritis nodosa. A diagnosis is based on the physical examination and a few laboratory studies. A patient is said to have polyarteritis nodosa if he or she has three of the ten following signs known as the 1990 ACR (American College of Rheumatology) criteria:

  • Unexplained weight loss greater than/equal to 8 lbs.
  • Purplish rash over extremities or torso (livedo reticularis)
  • Testicular pain or tenderness
  • Muscle pain, weakness or leg tenderness
  • Nerve disease
  • High blood pressure
  • Elevated kidney blood tests
  • Positive test for hepatitis B virus
  • Arteriogram showing dilated or constricted arteries
  • Biopsy of tissue showing inflamed arteries

What is the treatment for polyarteritis nodosa?

Treatment comprises medications to suppress the immune system, including prednisone and cyclophosphamide.

For PAN related to hepatitis, treatment may require plasmapheresis and antiviral medicines. Plasmapheresis is a procedure in which blood plasma is removed from the circulation and replaced by fluid, protein or donated plasma.

What is the prognosis for polyarteritis nodosa?

When treated, 90 percent of cases can be brought into remission or cured. If the disease is left untreated, complications involving the kidneys and gastrointestinal tract are usually severe.

Medication toxicity remains a major concern in Polyarteritis nodosa. Therefore, it is very important that patients get regular check-ups, be vaccinated and be constantly monitored for any infection or side effects of therapy. All patients affected by polyarteritis nodosa should be under the supervision of a skilled health team that is familiar with these conditions, their treatment and the side effects of immunosuppressive medications.
 





Contact Name
Rush Vasculitis Clinic
Contact Phone
(312) 563-2800
Contact E-mail
contact_rush@rush.edu



Location
Orthopedic Building
1611 W. Harrison St., Suite 510
Chicago, IL 60612



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