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Polyarteritis nodosa

Periarteritis Nodosa, Necrotizing Angitis, Disseminated Periarteritis, Infectious Periarteritis, Necrotizing Periarteritis

What is Periarteritis nodosa?

The disorder is characterized by inflammation and destruction of small and medium-size arteries in many organ systems. The inflammation can result in obstruction of the vessels that reduces the supply of blood reaching the affected area. The skin, intestines, kidney, liver, and heart are at greatest risk. Periarteritis nodosa is an uncommon disease, with age of onset usually between 40 and 60 years. The cause is unknown. The disease is associated with hepatitis B and C, lupus, and rheumatoid arthritis. The disease is more common in intravenous drug abusers than in the general population.

How is it diagnosed?

There is no single test that can confirm a diagnosis of polyarteritis nodosa (PAN). Instead, the physician relies on a variety of tests and observations that narrow the range of possibilities until a diagnosis of polyarteritis nodosa is confirmed.

The first step a physician will take in diagnosing PAN is to obtain a patient's personal and family medical history. For example, the physician will ask if the patient has ever been diagnosed with hepatitis B or C or leukemia. These diseases are frequently present in sufferers of PAN.

The physician will next give the patient a physical examination, focusing particularly on areas where the patient is having symptoms. A number of tests will be ordered to obtain additional information, including:

Blood tests. These tests can detect infections such as hepatitis B and C and can give a general assessment of how the kidneys are functioning. Other general blood tests such as a complete blood count and sedimentation rate can be used as a screening of someone's health. Additionally, levels of C reactive protein, an inflammatory marker, may be elevated.
Urinalysis. Analysis of a patient's urine sample can help determine the health of the patient's kidneys.
Biopsy. If there is skin, muscle or nerve involvement, the physician can use the analysis of affected tissue to diagnose PAN. If the death (infarction) of an organ is suspected, then the physician may need to take a tissue sample from that organ.
Chest x-ray. This may reveal abnormalities in the lungs and chest that are typical of PAN.
Arteriogram. A test used to image the inside of a blood vessel to check for aneurysms and other abnormalities. Aneurysms most often affect arteries leading to the kidneys, liver or gastrointestinal tract in PAN patients.
Coronary angiogram. If damage to the heart is suspected, the physician may order an angiogram. To perform this catheter-based test, a thin, hollow tube (catheter) is inserted through an artery (usually the femoral artery in the groin) and guided all the way to the heart. A special dye (contrast medium) is injected through the catheter and into the heart so that the angiogram (a type of x-ray) can be taken.
These tests can help a physician determine whether the patient meets at least three of the 10 criteria needed to diagnosis PAN. According to the American College of Rheumatology, these 10 criteria are:
  • Weight loss greater than or equal to 4 kilograms (about 9 pounds) since the illness began
  • Livedo reticularis (a type of skin discoloration)
  • Testicular pain or tenderness
  • Pain or weakness in the legs
  • Mononeuropathy or polyneuropathy (numbness or “pins and needles”)
  • Diastolic high blood pressure (hypertension)
  • Elevated BUN or creatinine (as measured by a blood test)
  • Presence of the hepatitis B antigen or antibody
  • Abnormal arteriogram
  • Abnormal biopsy of an artery

Treatment and prevention of polyarteritis nodosa

Treatment is essential for saving the life of a patient with polyarteritis nodosa (PAN). Untreated cases of PAN typically result in death, often by kidney failure, heart attack or heart failure within several years.

Treatment may depend on the underlying conditions that are present. Recent research has shown that if PAN is present with hepatitis B, antiviral therapy is effective, whereas the conventional treatment may actually worsen their viral disease.

For patients who do not have hepatitis or leukemia, physicians typically take a two-pronged approach. First, they will attempt to treat the inflammation of the blood vessels with powerful medications that reduce inflammation (corticosteroids). Second, medications that reduce the body's immune response have been shown to help relieve symptoms.

For patients with hypertension, physicians may prescribe ACE inhibitors. However, ACE inhibitors have been shown to worsen renal function, so physicians may instead prescribe calcium channel blockers. Both of these drugs are used to lower blood pressure.

Other conditions, such as kidney failure, heart failure or hepatitis, will be treated accordingly.

In severe cases, surgery may be necessary. For example, part of the patient's intestine may need to be removed if it was essentially killed by PAN. Kidney transplantation may also be necessary.

Since hepatitis B and C are considered risk factors for PAN, people are encouraged to protect themselves from these infections.

Medications

  • Cortisone drugs in high doses until acute symptoms diminish. Then symptoms may be controlled by a schedule of alternate-day cortisone.
  • Drugs to treat disorders of organs involved with this serious disease, such as heart medications for heart involvement or antihypertensives for high blood pressure.
  • Immunosuppressive drugs either alone or with steroids if other drugs fail. These drugs pose additional risks, including severe generalized bacterial infections.
Information
Brand
Generic
Label
Prednisone
Deltasone
Prednisone
Off-Label

Famvir (Famciclovir), Cytoxan (Cyclophosphamide)


What might complicate it?

Kidney involvement occurs eventually in more than 80% of cases. Inflammation of the gallbladder (cholecystitis) and appendix (appendicitis) can occur. Cardiac disorders such as inflammation of the heart muscle (myocarditis), its covering membrane (pericarditis), or rhythm disturbances occur in the late stages of the disease. The most frequent causes of death are gastrointestinal hemorrhage and perforation, congestive heart failure, and infections. There can be complications from drug therapy because it suppresses the immune system. The individual has a higher risk of developing an infection or cancer.

Predicted outcome

Corticosteroid therapy usually results in decreased severity or disappearance of the symptoms, but impaired kidney function or nerve damage can persist. Currently, the five-year survival rate is 50% to 90%, depending on the treatment given. Without treatment, five-year survival is five to ten percent.

Alternatives

Other disorders that also involve inflammation of the arteries are lupus, rheumatoid arthritis, rheumatic fever, serum sickness, or bacterial sepsis.

Appropriate specialists

Rheumatologist, gastroenterologist, dermatologist, cardiologist and nephrologist.

Last updated 8 July 2021