Polyarteritis Nodosa

Definition

Definition of Polyarteritis Nodosa
Polyarteritis nodosa (also known as "Panarteritis nodosa," and "Periarteritis nodosa") is a vasculitis of medium & small-sized arteries, which become swollen and damaged from attack by rogue immune cells. Polyarteritis nodosa is also called Kussmaul disease or Kussmaul-Maier disease. Infantile polyarteritis nodosa is a type of PAN restricted to infants.

Symptoms

Symptoms of Polyarteritis Nodosa
Symptoms result from damage to affected organs, often the skin, heart, kidneys, and nervous system.

Generalized symptoms include:

1. Abdominal pain
2. Decreased appetite
3. Fatigue
4. Fever
5. Joint aches
6. Muscle aches
7. Unintentional weight loss
8. Weakness

Causes

Causes of Polyarteritis Nodosa
Polyarteritis nodosa is a disease of unknown cause that affects arteries, the blood vessels that carry blood from the heart to organs and tissues. A minority of people (about 30%) diagnosed have an active Hepatitis B infection, and men are also twice as likely to get the disease as women.

Diagnosis

Diagnosis of Polyarteritis Nodosa
There are no specific lab tests for diagnosing polyarteritis nodosa. Diagnosis is based on the physical examination and a few laboratory studies that help to confirm the diagnosis:

1. Arteriogram to see any changes in the smaller arteries
2. CBC may show a higher than normal white blood count
3. ESR or CRP is often higher than normal
4. Tissue biopsy reveals inflammation in small arteries, called arteritis
5. Immunoglobulins may be increased

Treatment

Treatment of Polyarteritis Nodosa
Treatment involves medications to suppress the immune system, including prednisone and cyclophosphamide.

For polyarteritis nodosa related to hepatitis, treatment may involve plasmapheresis and anti-viral medicines.

Prognosis

Prognosis of Polyarteritis Nodosa
Current treatments using steroids and other drugs that suppress the immune system (such as cyclophosphamide) can improve symptoms and the chance of long-term survival. The most serious associated conditions generally involve the kidneys and gastrointestinal tract. Without treatment, the outlook is poor.

Prevention

Prevention of Polyarteritis Nodosa
There is no known prevention. However, early treatment can prevent some damage and symptoms.