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?
History: The majority of individuals will have nonspecific symptoms of generally feeling ill (malaise), fatigue, weight loss, headache, and abdominal pain. Joint and muscle pain, particularly in the calves, is a common complaint. Difficulty breathing and wheezing can be a symptom of non-classical polyarteritis nodosa. The general nature of these symptoms can make diagnosis difficult until late in the disease when symptoms of extreme high blood pressure, acute abdomen (surgical emergency), stroke, or nerve damage are reported.
Physical exam: Physical findings are nonspecific. Hypertension may be present. Skin abnormalities are observed in many individuals, the most frequent of which is bluish mottled skin of the legs and hands (livedo reticularis). The arteries of the retina may show changes. There may be wheezing heard in the lungs.
Tests: Blood tests are abnormal but not specific to this disease. Abnormalities include anemia (elevated white blood cell count and platelet count) and elevated sedimentation rate. Urinalysis typically shows protein and red blood cells and casts. Surgical removal of a piece of affected tissue (biopsy) and microscopic examination can make a definitive diagnosis when the inflammatory changes are seen in the arteries. Examination of arteries by x-rays (angiography) can reveal characteristic changes in the kidney, liver, or intestine.
How is it treated?
No cure exists for periarteritis nodosa. Initial treatment is high doses of steroids, with gradual tapering of the dose after response is observed. In more severe cases, a drug may be added that suppresses the body's immune response.
- 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.
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.
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.
Rheumatologist, gastroenterologist, dermatologist, cardiologist and nephrologist.
Last updated 27 May 2012