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Polyarteritis nodosa

Definition

Polyarteritis nodosa is a serious inflammatory blood vessel disease. The small and medium-sized arteries become swollen and damaged.

Alternative Names

Periarteritis nodosa; PAN; Systemic necrotizing vasculitis

Causes

Arteries are the blood vessels that carry oxygen-rich blood to organs and tissues. The cause of polyarteritis nodosa is unknown. The condition occurs when certain immune cells attack the affected arteries. The tissues that are fed by the affected arteries do not get the oxygen and nourishment they need. Damage occurs as a result.

More adults than children get this disease.

People with active hepatitis B or hepatitis C may develop this disease.

Angina causes and symptoms
Angina, or angina pectoris, is temporary chest pain or a sensation of pressure that occurs when the heart’s need for oxygen is not satisfied by the amount of blood supplied to the heart. Blood flow can be limited when the arteries are narrowed by atherosclerosis, or because of coronary artery spasms. Symptoms of angina may also include discomfort in the shoulders, inside the arms, through the back and in the throat, jaw, or teeth. Angina due to atherosclerosis usually occurs during physical exertion or emotional distress, or at rest if the artery is narrowed by more than 70%.

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:

If nerves are affected, you may have numbness, pain, burning, and weakness. Damage to the nervous system may cause strokes or seizures.

Exams and Tests

No specific lab tests are available to diagnose polyarteritis nodosa. There are a number of disorders that have features similar to polyarteritis 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

Treatment involves medicines to suppress inflammation and the immune system. These may include steroids, such as prednisone. Similar medicines, such as azathioprine, methotrexate or mycophenolate that allow for reducing the dose of steroids are often used as well. Cyclophosphamide is used in severe cases.

For polyarteritis nodosa related to hepatitis, treatment may involve plasmapheresis and antiviral medicines.

Outlook (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.

Possible Complications

Complications may include:

When to Contact a Medical Professional

Contact your health care provider if you develop symptoms of this disorder. Early diagnosis and treatment may improve the chance of a good outcome.

Prevention

There is no known prevention. However, early treatment can prevent some damage and symptoms.

Gallery

Angina causes and symptoms
Angina, or angina pectoris, is temporary chest pain or a sensation of pressure that occurs when the heart’s need for oxygen is not satisfied by the amount of blood supplied to the heart. Blood flow can be limited when the arteries are narrowed by atherosclerosis, or because of coronary artery spasms. Symptoms of angina may also include discomfort in the shoulders, inside the arms, through the back and in the throat, jaw, or teeth. Angina due to atherosclerosis usually occurs during physical exertion or emotional distress, or at rest if the artery is narrowed by more than 70%.
Microscopic polyarteritis 2
Polyarteritis nodosa, seen here on a lower extremity, produces painful, erythematous nodules. Arteries in the deep dermis and upper fat show neutrophilic infiltration.
Circulatory system
Blood used by the body is brought back to the heart and lungs by the veins of the body. Once the blood has gathered more oxygen from the lungs, it is pumped back out to the body through the arteries.

References

Luqmani R, Awisat A. Polyarteritis nodosa and related disorders. In: Firestein GS, Budd RC, Gabriel SE, Koretzky GA, McInnes IB, O'Dell JR, eds. Firestein & Kelley's Textbook of Rheumatology. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 95.

Puéchal X, Pagnoux C, Baron G, et al. Adding azathioprine to remission-induction glucocorticoids for eosinophilic granulomatosis with polyangiitis (Churg-Strauss), microscopic polyangiitis, or polyarteritis nodosa without poor prognosis factors: a randomized, controlled trial. Arthritis Rheumatol. 2017;69(11):2175-2186. PMID: 28678392 www.pubmed.ncbi.nlm.nih.gov/28678392/.

Shanmugam VK. Vasculitis and other uncommon arteriopathies. In: Sidawy AN, Perler BA, eds. Rutherford's Vascular Surgery and Endovascular Therapy. 10th ed. Philadelphia, PA: Elsevier; 2023:chap 138.

Stone JH. The systemic vasculitides. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 254.

Last reviewed April 19, 2022 by Diane M. Horowitz, MD, Rheumatology and Internal Medicine, Northwell Health, Great Neck, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team..

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