Polyarteritis nodosa
Polyarteritis nodosa[edit | edit source]
Alternate names - PAN; Periarteritis; Polyarteritis
Definition[edit | edit source]
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
- 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[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:
- 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[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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