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Polyarteritis nodosa

polyarteritis nodosa, polyarteritis nodosa symptoms
Polyarteritis nodosa PAN, also known as panarteritis nodosa, periarteritis nodosa, Kussmaul disease, or Kussmaul-Maier disease, is a systemic necrotizing inflammation of blood vessels vasculitis affecting small- or medium-sized muscular arteries, typically involving the arteries of the kidneys and other internal organs but generally sparing the lungs' circulation Polyarteritis nodosa may present in infants In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary, therefore making "rosary sign" an important diagnostic feature of the vasculitis PAN is associated with hepatitis B virus infection

Since hepatitis B virus infection is becoming less common, PAN has become increasingly rare With treatment, five-year survival is 80%; without treatment, five-year survival is 13% Death is often a consequence of kidney failure, myocardial infarction, or stroke


  • 1 Signs and symptoms
  • 2 Causes
  • 3 Diagnosis
    • 31 Differential diagnosis
  • 4 Treatment
  • 5 Epidemiology
  • 6 History
  • 7 In popular media
  • 8 References
  • 9 External links

Signs and symptoms

PAN may affect nearly every organ system and thus it can present with a broad array of signs and symptoms These manifestations result from ischemic damage to affected organs, often the skin, heart, kidneys, and nervous system Constitutional symptoms are seen in up to 90% of affected individuals and include fever, fatigue, weakness, loss of appetite, and unintentional weight loss Muscle and joint aches are common The skin may show rashes, swelling, necrotic ulcers, and subcutaneous nodules lumps Palpable purpura and livedo reticularis can occur in some patients

Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness peripheral neuropathy Peripheral nerves are often affected and this most commonly presents as mononeuritis multiplex, which is the most common neurologic sign of PAN Central nervous system involvement may cause strokes or seizures Kidney involvement is common and often leads to death of parts of the kidney though kidney filtration function is often preserved Involvement of the renal artery, which supplies the kidneys with highly oxygenated blood, often leads to high blood pressure in about one-third of cases deposition of protein or blood in the urine may also be seen Involvement of the arteries of the heart may cause a heart attack, heart failure, and inflammation of the sac around the heart pericarditis


There is no association with ANCA, but about 30% of people with PAN have chronic hepatitis B and deposits containing HBsAg-HBsAb complexes in affected blood vessels, indicating an immune complex–mediated cause in that subset Infection with the Hepatitis C virus and HIV are occasionally discovered in people affected by PAN The cause remains unknown in the remaining cases; there may be causal and clinical distinctions between classic idiopathic PAN, the cutaneous forms of PAN, and PAN associated with chronic hepatitis In children, cutaneous PAN is frequently associated with streptococcal infections, and positive streptococcal serology is included in the diagnostic criteria


Microscopic findings in polyarteritis nodosa: nodular thickened and branched arteries from small bowel mucosa Fig 1, flexor digitorum superficialis artery with early diffuse nuclear proliferation X155; Fig 2, nodular thickened and aneurysmal expanded artery: a tunica intima, b tunica media, c tunica adventitia, d newly formed connective tissue and fat Fig 3; X155

No specific lab tests exist for diagnosing polyarteritis nodosa Diagnosis is generally based on the physical examination and a few laboratory studies that help confirm the diagnosis:

  • CBC may demonstrate an elevated white blood count
  • ESR elevated
  • Perinuclear pattern of antineutrophil cytoplasmic antibodies p-ANCA - not associated with "classic" polyarteritis nodosa, but is present in a form of the disease affecting smaller blood vessels, known as microscopic polyangiitis or leukocytoclastic angiitis
  • Tissue biopsy reveals inflammation in small arteries, called arteritis
  • Elevated C-reactive protein

A patient is said to have polyarteritis nodosa if he or she has three of the 10 signs known as the 1990 American College of Rheumatology ACR criteria, when a radiographic or pathological diagnosis of vasculitis is made:

  • Weight loss greater than/equal to 45 kg
  • Livedo reticularis a mottled purplish skin discoloration over the extremities or torso
  • Testicular pain or tenderness occasionally, a site biopsied for diagnosis
  • Muscle pain, weakness, or leg tenderness
  • Nerve disease either single or multiple
  • Diastolic blood pressure greater than 90 mmHg high blood pressure
  • Elevated kidney blood tests BUN greater than 40 mg/dL or creatinine greater than 15 mg/dL
  • Hepatitis B not C virus tests positive for surface antigen or antibody
  • Arteriogram angiogram showing the arteries that are dilated aneurysms or constricted by the blood vessel inflammation
  • Biopsy of tissue showing the arteritis typically inflamed arteries: The sural nerve is a frequent location for the biopsy
In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary, therefore making "rosary sign" a diagnostic feature of the vasculitis

In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary, therefore making "rosary sign" an important diagnostic feature of the vasculitis The 1990 ACR criteria were designed for classification purposes only Nevertheless, their good discriminatory performances, indicated by the initial ACR analysis, suggested their potential usefulness for diagnostic purposes as well Subsequent studies did not confirm their diagnostic utility, demonstrating a significant dependence of their discriminative abilities on the prevalence of the various vasculitides in the analyzed populations Recently, an original study, combining the analysis of more than 100 items used to describe patients' characteristics in a large sample of vasculitides with a computer simulation technique designed to test the potential diagnostic utility of the various criteria, proposed a set of eight positively or negatively discriminating items to be used as a screening tool for diagnosis in patients suspected of systemic vasculitis

Differential diagnosis

Polyarteritis nodosa rarely affects the blood vessels of the lungs and this feature can help to differentiate it from other vasculitides, which may have similar signs and symptoms eg, granulomatosis with polyangiitis or microscopic polyangiitis


Treatment involves medications to suppress the immune system, including prednisone and cyclophosphamide Underlying hepatitis B virus infection should be immediately treated In some cases, methotrexate or leflunomide may be helpful Some patients have also noticed a remission phase when a four-dose infusion of rituximab is used before the leflunomide treatment is begun Therapy results in remissions or cures in 90% of cases Untreated, the disease is fatal in most cases The most serious associated conditions generally involve the kidneys and gastrointestinal tract A fatal course usually involves gastrointestinal bleeding, infection, myocardial infarction, and/or kidney failure

In case of remission, about 60% experience relapse within five years In cases caused by hepatitis B virus, however, recurrence rate is only around 6%


The condition affects adults more frequently than children and males more frequently than females Most cases occur between the ages of 40 and 60 Polyarteritis nodosa is more common in people with hepatitis B infection


The medical eponyms Kussmaul disease or Kussmaul-Maier disease reflect the seminal description of the disease in the medical literature by Adolph Kussmaul and Rudolf Robert Maier

In popular media

The 1956 film Bigger Than Life featured the protagonist being diagnosed with polyarteritis nodosa


  1. ^ a b Rapini, Ronald P; Bolognia, Jean L; Jorizzo, Joseph L 2007 Dermatology: 2-Volume Set St Louis: Mosby ISBN 1-4160-2999-0 
  2. ^ synd/764 at Who Named It
  3. ^ a b Kumar, Vinay; K Abbas, Abul; C Aster, Jon 2015 Robbins and Cotran: Pathologic Basis of Disease 9th ed Elsevier p 509 ISBN 978-1-4557-2613-4 
  4. ^ Person, A, Donald 2006-06-15 "Infantile Polyarteritis Nodosa" eMedicine WebMD Retrieved 24 December 2009 
  5. ^ a b Keen, William Surgery, Volume 5 W B Saunders p 243 
  6. ^ a b Greenson, Joel K; Montgomery, Elizabeth A; Polydorides, Alexandros D 1 September 2009 Diagnostic Pathology: Gastrointestinal: Published by Amirsys Lippincott Williams & Wilkins ISBN 978-1-931884-26-6 Retrieved 19 August 2013 
  7. ^ a b c d e f g h i j k l m n o p q Forbess, L; Bannykh, S 2015 "Polyarteritis Nodosa" Rheumatic Disease Clinics of North America 41 1: 33-46 doi:101016/jrdc201409005 PMID 25399938 
  8. ^ Russell Goodman; Paul F Dellaripa; Amy Leigh Miller; Joseph Loscalzo January 2, 2014 "An Unusual Case of Abdominal Pain" N Engl J Med 370 1: 70–75 doi:101056/NEJMcps1215559 
  9. ^ Sarah Ringold; Carol A Wallace May 1, 2010 "Evolution of paediatric-specific vasculitis classification criteria" Annals of the Rheumatic Diseases 69: 785–86 doi:101136/ard2009127886 
  10. ^ American College of Rheumatology
  11. ^ Shiel, Jr, William C, http://wwwmedicinenetcom/polyarteritis_nodosa/articlehtm
  12. ^ Henegar, Corneliu; Pagnoux, Christian; Puéchal, Xavier; Zucker, Jean-Daniel; Bar-Hen, Avner; Guern, Véronique Le; Saba, Mona; Bagnères, Denis; Meyer, Olivier; Guillevin, Loïc 1 May 2008 "A paradigm of diagnostic criteria for polyarteritis nodosa: Analysis of a series of 949 patients with vasculitides" Arthritis & Rheumatism 58 5: 1528–1538 doi:101002/art23470 PMID 18438816 
  13. ^ Boehm, Ingrid; Bauer, R 1 February 2000 "Low-Dose Methotrexate Controls a Severe Form of Polyarteritis Nodosa" Archives of Dermatology 136 2: 167–9 doi:101001/archderm1362167 PMID 10677090 
  14. ^ Giannini, AJ; Black, HR Psychiatric, Psychogenic and Somatopsychic Disorders Handbook Garden City, NY Medical Examination Publishing, 1978 Pp 219–220 ISBN 0-87488-596-5
  15. ^ Selga, D; Mohammad, A; Sturfelt, G; Segelmark, M 2006 "Polyarteritis nodosa when applying the Chapel Hill nomenclature--a descriptive study on ten patients" Rheumatology 45 10: 1276–1281 doi:101093/rheumatology/kel091 PMID 16595516 , Entry in Polyarteritis Nodosa Follow-up article
  16. ^ Guillevin, L; Lhote, F; Cohen, P; Sauvaget, F; Jarrousse, B; Lortholary, O; Noël, L; Trépo, C 1995 "Polyarteritis nodosa related to hepatitis B virus A prospective study with long-term observation of 41 patients" Medicine 74 5: 238–253 doi:101097/00005792-199509000-00002 PMID 7565065  , Entry in Polyarteritis Nodosa Follow-up article

External links

Classification V · T · D
  • ICD-10: M300
  • ICD-9-CM: 4460
  • MeSH: D010488
  • DiseasesDB: 10220
External resources
  • MedlinePlus: 001438

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Polyarteritis nodosa

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