Polyarteritis nodosa

Polyarteritis nodosa

Alternate names - PAN; Periarteritis; Polyarteritis

Definition

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

• 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

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

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

• Abnormality of the kidney(Abnormal kidney)
• Arthralgia(Joint pain)
• Elevated C-reactive protein level
• Fever
• Myalgia(Muscle ache)
• Polyneuritis
• Weight loss

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

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:

• Complete blood count (CBC) with differential, creatinine, tests for hepatitis B and C, and urinalysis
• Erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP)
• Serum protein electrophoresis, cryoglobulins
• Serum complement levels
• Arteriogram
• Tissue biopsy
• Other blood tests will be done to rule out similar conditions, such as systemic lupus erythematosus (ANA) or granulomatosis with polyangiitis (ANCA)
• Test for HIV
• Cryoglobulins
• Anti-phospholipid antibodies
• Blood cultures

Treatment

• 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

• 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

• NIH info on Polyarteritis nodosa

Polyarteritis nodosa is a rare disease.

Polyarteritis nodosa Resources
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