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Polyarteritis nodosa

This is part of Rare diseases.

Diagnosis: Polyarteritis nodosa

Synonyms: PAN

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Date of publication: 2014-12-10
Version: 2.0

ICD 10 code

M30.0

The disease

Polyarteritis nodosa (PAN) is a form of vasculitis, a group of diseases characterised by inflammation of the body's medium-sized arteries. Blood circulation deteriorates and blood vessels may dilate or burst. Symptoms are caused by damage to organs including the nerves, muscles, intestines and kidneys.

The term polyarteritis nodosa (poly = many, arteritis = arterial inflammation, nodus = lump) has been used for more than a century to describe different conditions associated with extensive inflammation of the blood vessels. Over time, specific diagnoses within this group of inflammatory diseases have been identified and named, so the term has narrowed. Nevertheless the parameters defining polyarteritis nodosa are still unclear. The first descriptions of the disease were made in 1866 by the German physicians Adolph Kussmaul and Rudolf Robert Maier. There is also a form of the disease which affects the skin, known as cutaneous polyarteritis nodosa (CPAN).

Occurrence

Studies from different countries show the number of people developing the disease as between 0.1 and 1 per 100,000 annually. In Sweden it is 0.1 per 100,000, meaning that approximately ten people become ill every year. The occurrence in Sweden is difficult to estimate, but there are probably approximately 300 people in the country with the disease. It is found equally in men and women.

Cause

The cause of the disease is unknown. It is characterized by patches of inflammation in the walls of medium-sized arteries. Examination of the affected tissues shows the presence of inflammatory cells. This inflammation causes parts of the walls of the blood vessels to weaken, while nodular aneurysms (bulges) form along the arteries. There is a risk that these aneurysms burst and cause bleeding.

There are no diagnostic, autoantibody tests for polyarteritis nodosa. Autoantibodies, are antibodies directed against the body itself. In France approximately half of those with the disease also have hepatitis B (liver inflammation and jaundice caused by a virus).

Heredity

No pattern of inheritance has been shown to be involved.

Symptoms

The most common age for the disease to manifest is between 40 and 60. It is extremely rare in children, but if it does present in childhood it is usually at between the ages of nine and eleven. If treatment commences early, survival rates are 80 per cent and there are often long periods of remission.

The first symptoms are a general feeling of malaise, fatigue, fever, aching muscles and joints, and weight loss. The renal arteries are often affected and blood pressure rises. There is a risk of renal failure. Other symptoms sometimes include blood in the urine (haematuria) and high levels of protein in the urine (proteinuria). Kidney failure does not give rise to early symptoms but over time swelling and nausea may develop, and the quantity of urine diminishes.

The gastrointestinal tract is usually affected causing abdominal pain, nausea and vomiting. The intestine may bleed or burst, causing peritonitis. Pancreatitis may also develop, causing pain in the upper part of the stomach.

The peripheral nerves are often affected (neuropathy) as a result of inflammation in the blood vessels supplying the nerves. The peripheral part of the nervous system does not belong to the brain or spinal cord. Neuropathy is commonly asymmetric, affecting individual nerves (mononeuritis multiplex). Symptoms include persistent numbness, tingling or pain in the feet and/or hands, often combined with reduced muscle strength. The nerve controlling the muscles which lift the foot may be damaged (the peroneus nerve) resulting in foot drop.

In very rare cases the central nervous system (brain and spinal cord) are affected by a temporary disturbance in the flow of blood to the brain. This is known as a transient ischemic attack (TIA) and, regardless of severity, symptoms disappear within 24 hours.

Heart disease may develop, including angina, heart attack and heart failure.

Men with the disease may develop testicular inflammation.

Skin abnormalities are common in polyarteritis nodosa, above all on the legs. Small, tender lumps under the skin, blisters, small and/or wide-spread lesions and mottled, purplish discoloration (livedo reticularis) are common. There is a form of the disease called cutaneous polyarteritis nodosa. This affects only the skin and not the inner organs.

Diagnosis

As the disease is so rare and symptoms vary so much, it is difficult to diagnose polyarteritis nodosa. There are no specific tests to confirm the diagnosis so it is made by noting signs and symptoms, and with the help of a detailed case history. Ordinary blood tests often show elevated sedimentation rates (SR) and anaemia as signs of general inflammation. Kidney function may be affected, which can manifest as blood and protein in the urine.

A CAT scan (computed tomography) and an MRI scan (magnetic resonance imaging) can identify blood vessels in those organs suspected of being affected. Small bulges (aneurysms) and minor bleeding support the diagnosis, but to confirm it a tissue sample (biopsy) is necessary. An examination of the tissue will show inflammation in the entire wall of the blood vessel, injury to or dilation of the blood vessel, or blood clots.

In 1990, classification criteria were published by the American College of Rheumatology (ACR). At least three of the following criteria shall be met if the condition is to be confirmed as polyarteritis nodosa:

  • Loss of weight
  • Changes in the skin (livedo reticularis)
  • Tenderness in the testicles in men
  • Muscle tenderness
  • Nerve disorders associated with persistent numbness, tingling and pain
  • Elevated blood pressure
  • Kidney damage
  • Hepatitis B
  • Abnormal results from angiography
  • Tests on tissue showing inflammation of the walls of the blood vessels.

There are signs or symptoms which make the diagnosis unlikely: lung problems, certain antibodies present in a blood test (ANCA) or glomerulonephritis (inflammation of the small blood vessels of the kidney). In such cases another inflammatory disease of the blood vessels is more probable.

Treatment/interventions

People who have received the diagnosis polyarteris nodosa should be examined and treated by, or by a physician collaborating with, a rheumatologist. It is important that treatment starts as early as possible and that the progression of the disease and its treatment are regularly monitored. Basic treatment takes the form of cortisone tablets. In a milder form of the disease in which the kidneys, gastro-intestinal tract and nervous system are not affected, this can be sufficient. Treatment is always long-term and usually extends over a year.

Often cortisone treatment is not sufficient and chemotherapy may be necessary. Cyclophosphamide is the first choice of medication to prevent damage to affected organs. In certain severe cases both cortisone and cyclophosphamide need to be administered intravenously during the initial period of treatment. Medication may also be given to counteract or prevent the most common side effects of cortisone treatment, including osteoporosis and stomach ulcers. If a person has both a hepatitis B infection (jaundice) and polyarteritis nodosa both diseases need to be treated simultaneously. Long-term monitoring is essential to check that symptoms do not recur.

Regular check-ups offer important opportunities to discuss how the disease effects the work situation, leisure activities and family life. It is important that information is available to the person affected as well as friends and family. Psychological and social support may also be necessary.

Practical advice

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National and regional resources in Sweden

Resources include rheumatology departments at Sweden’s regional and university hospitals.

Resource personnel

Dr Iva Gunnarsson, Rheumatology Department, Karolinska University Hospital, Huddinge, SE-141 86 Stockholm, Sweden. Tel: +46 8 585 800 00, email: iva.gunnarsson@karolinska.se.

Dr Ann Knight, Rheumatology Department, Uppsala University Hospital, SE-751 85 Uppsala, Sweden. Tel: +46 18 611 92 29, email: ann.knight@medsci.uu.se.

Professor Mårten Segelmark, Renal Department, Linköping University Hospital, SE-581 85 Linköping, Sweden. Tel: +46 13 22 20 00, email: marten.segelmark@lio.se.

Courses, exchanges of experience, recreation

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Organizations for the disabled/patient associations etc.

The Swedish Rheumatism Association, Alströmergatan 39, Stockholm. Mailing address: Box 12851, SE-112 98 Stockholm, Sweden. Tel: +46 8 505 805 00, fax: +46 8 505 805 50, email: info@reumatikerforbundet.org, www.reumatikerforbundet.org.

There is a smaller association for people with ANCA-associated vasculitis (AAV) within the Swedish Rheumatism Association: www.smavaskulit.reumatikerforbundet.org.

Courses, exchanges of experience for personnel

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Research and development

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Information material

Short summaries of all the database texts are available as leaflets, in Swedish only. These leaflets may be ordered or printed out. (See under "Mer hos oss" in the right hand column.)

Literature

Kussmaul A , Maier R. Über eine bisher nicht beschriebene eigentümlichen Arterienerkrankung (Periarteritis nodosa) die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskellähmung einhergeht. Dtsch Arch Klin Med 1866; 1: 484-517.

Mohammad AJ, Jacobsson LTH, Mahr AD, Sturfelt G, Segelmark M. Prevalence of Wegener’s granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg–Strauss syndrome within a defined population in southern Sweden. Rheumatology 2007; 46: 1329-1337.

Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum 2010; 62: 616-626.

Pettigrew HD, Teuber SS, Gershwin ME. Polyarteritis nodosa. Compr Ther 2007; 33: 144-149.

Segelmark M, Selga D. The challenge of managing patients with polyarteritis nodosa. Curr Opin Rheumatol 2007; 19: 33-38.

Books

Bratt J, Knight A. Polyarteritis nodosa. Reumatologi. (In Swedish only.) Ed: Klareskog L, Saxne T, Enman Y. Studentlitteratur, Lund 2011; 213.

Database references

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Document information

The Swedish Information Centre for Rare Diseases produced and edited this information material.

The medical expert who wrote the draft of this information material is Senior Physician Ann Knight, Uppsala University Hospital, Sweden.

The relevant organizations for the disabled/patient associations have been given the opportunity to comment on the content of the text.

An expert group on rare diseases, affiliated with the University of Gothenburg, approved the material prior to publication.

Date of publication: 2014-12-10
Version: 2.0
Date of publication of the Swedish version: 2014-06-09

For enquiries contact The Swedish Information Centre for Rare Diseases, The Sahlgrenska Academy at the University of Gothenburg, Box 422, SE-405 30 Gothenburg, Sweden. Tel: +46 31 786 55 90, email: ovanligadiagnoser@gu.se.

 

About the database

This knowledge database provides information on rare diseases and conditions. The information is not intended to be a substitute for professional medical care, nor is it intended to be used as a basis for diagnosis or treatment.