Polyarteritis nodosa

From WikiMD's Wellness Encyclopedia

(Redirected from Infantile polyarteritis)

Polyarteritis nodosa[edit | edit source]

Alternate names - PAN; Periarteritis; Polyarteritis

Definition[edit | edit source]

Kussmaul Maier Makro.jpg

Polyarteritis nodosa (PAN) is a blood vessel disease characterized by inflammation of small and medium-sized arteries (vasculitis), preventing them from bringing oxygen and food to organs.

Cause[edit | edit source]

  • The exact cause of polyarteritis nodosa (PAN) is not known, and in most cases, no predisposing cause has been found (it is idiopathic).
  • Many scientists believe that it is an autoimmune disease.
  • Research has suggested that an abnormal immune response to an initial infection may trigger the development of PAN.
  • However, the reasons that many smaller arteries and capillaries are spared is not understood.
  • Hepatitis B virus (HBV), hepatitis C, and hairy cell leukemia have been associated with some cases of PAN.
  • In one report from France, HBV accounted for a third of the cases of PAN.
  • HBV-related PAN typically occurs within four months after the onset of HBV infection.
  • PAN has also been seen in drug abusers (particularly those using amphetamines).
  • It has also appeared to occur as an allergic reaction to some drugs and vaccines.
  • The specific symptoms of PAN are due to ischemia or infarction of tissues and organs.
  • Thickening of the walls of affected vessels causes narrowing of the inside of the vessels, reducing blood flow and predisposing to blood clots in affected vessels.

Signs and symptoms[edit | edit source]

Symptoms are caused by damage to affected organs. The skin, joints, muscle, gastrointestinal tract, heart, kidneys, and nervous system are often affected.

Symptoms include:

  • Abdominal pain
  • Decreased appetite
  • Fatigue
  • Fever
  • Joint aches
  • Muscle aches
  • Unintentional weight loss
  • Weakness
  • If nerves are affected, you may have numbness, pain, burning, and weakness. Damage to the nervous system may cause strokes or seizures.

Clinical presentation[edit | edit source]

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

30%-79% of people have these symptoms

5%-29% of people have these symptoms

  • Abdominal pain(Pain in stomach)
  • Abnormality of the gastrointestinal tract
  • Cutis marmorata
  • Erythema
  • Hypertension
  • Morphological central nervous system abnormality
  • Pericarditis(Swelling or irritation of membrane around heart)
  • Raynaud phenomenon
  • Sensory axonal neuropathy
  • Skin ulcer(Open skin sore)
  • Subcutaneous nodule(Firm lump under the skin)

1%-4% of people have these symptoms

  • Abnormality of the eye(Abnormal eye)
  • Cardiomyopathy(Disease of the heart muscle)
  • Pleuritis(Inflammation of tissues lining lungs and chest)

Diagnosis[edit | edit source]

No specific lab tests are available to diagnose polyarteritis nodosa. There are a number of disorders that have features similar to polyarthritis nodosa. These are known as "mimics."

You will have a complete physical exam.

Lab tests that can help make the diagnosis and rule out mimics include:

Treatment[edit | edit source]

  • There is no cure for polyarteritis nodosa (PAN), but the disease and its symptoms can be managed.
  • The goal of treatment is to prevent disease progression and further organ damage.
  • The exact treatment depends on the severity in each person.
  • While many people do well with treatment, relapses can occur.
  • When the cause of PAN is unknown (idiopathic), treatment involves corticosteroids and immunosuppressive medications.
  • If there are no serious neurologic, renal, gastrointestinal, or heart symptoms, corticosteroids may initially be sufficient.
  • For severe disease with these symptoms, cyclophosphamide may also be used.
  • Hypertension should be treated aggressively.
  • When PAN is related to hepatitis B, treatment often involves steroids, anti-viral medications and sometimes plasma exchange (also called plasmapheresis).

Prognosis[edit | edit source]

  • Current treatments with steroids and other drugs that suppress the immune system (such as azathioprine or cyclophosphamide) can improve symptoms and the chance of long-term survival.
  • The most serious complications most often involve the kidneys and gastrointestinal tract.
  • Without treatment, the outlook is poor.


NIH genetic and rare disease info[edit source]

Polyarteritis nodosa is a rare disease.


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