Polyarteritis nodosa pathophysiology

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Polyarteritis nodosa Microchapters


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Differentiating Polyarteritis nodosa from other Diseases

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Ali Poyan Mehr, M.D. [2] Associate Editor(s)-in-Chief: Olufunmilola Olubukola M.D.[3]Cafer Zorkun, M.D., Ph.D. [4]Sargun Singh Walia M.B.B.S.[5]; Haritha Machavarapu, M.B.B.S.


The exact pathogenesis of PAN is not fully understood.It is a disease of unknown cause that affects arteries, the blood vessels that carry oxygenated blood to organs and tissues.PAN affects the medium sized arterial vessels.PAN does not affect large arterial vessels, capillaries, small arterioles and venous system. It occurs when certain immune cells attack the affected arteries.Inflammation starts in the vessel intima and results in fibrinoid necrosis by destroying the internal and external elastic lamina.Aneurysms and thrombi may develop at the site of lesions.One hypothesis is that this condition is caused by antibodies against HBV, via a type IIII hypersensitivity reaction.Due to inflammation of the medium to small sized vessels in PAN and the presence of impaired endothelial function, there could be direct endothelial cell activation and damage resulting from proinflammatory cytokines or antibodies (anti-endothelial cells antibodies).Mutation in CECR1 can lead to vascular and inflammatory disorders which also include PAN.




  • The development of PAN is the result of multiple genetic mutations.
    • Mutation in CECR1 can lead to vascular and inflammatory disorders which also include PAN.[6] [7]
      • CECR1 is also known as ADA2.
      • It is responsible for production of adenosine deaminase 2.

Associated Conditions

The following conditions are associated with the development of polyarteritis nodosa:

  • Hepatitis B infection
  • Hepatitis C infection

Microscopic Pathology

  • Microscopic histopathological analysis of a biopsy sample from a patient of PAN reveals:
    • Focal necrotizing arteritis
      • Mixed cellular infiltrate are seen within the vessel wall.
    • Nerve biopsy
      • Axonal degeneration
      • Fiber loss
      • Wallerian degeneration
      • Perineural necrosis
      • Neoangiogenesis around epineurium or perineurium
    • Segmental demyelination[8]


  1. Stone JH (October 2002). "Polyarteritis nodosa". JAMA. 288 (13): 1632–9. PMID 12350194.
  2. Stone JH (October 2002). "Polyarteritis nodosa". JAMA. 288 (13): 1632–9. PMID 12350194.
  3. Colmegna I, Maldonado-Cocco JA (August 2005). "Polyarteritis nodosa revisited". Curr Rheumatol Rep. 7 (4): 288–96. PMID 16045832.
  4. Saadoun D, Terrier B, Semoun O; et al. (2011). "Hepatitis C virus-associated polyarteritis nodosa". Arthritis Care Res (Hoboken). 63 (3): 427–35. doi:10.1002/acr.20381. PMID 20981809. Unknown parameter |month= ignored (help)
  5. Filer AD, Gardner-Medwin JM, Thambyrajah J, Raza K, Carruthers DM, Stevens RJ; et al. (2003). "Diffuse endothelial dysfunction is common to ANCA associated systemic vasculitis and polyarteritis nodosa". Ann Rheum Dis. 62 (2): 162–7. PMC 1754444. PMID 12525387.
  6. Zhou Q, Yang D, Ombrello AK, Zavialov AV, Toro C, Zavialov AV, Stone DL, Chae JJ, Rosenzweig SD, Bishop K, Barron KS, Kuehn HS, Hoffmann P, Negro A, Tsai WL, Cowen EW, Pei W, Milner JD, Silvin C, Heller T, Chin DT, Patronas NJ, Barber JS, Lee CC, Wood GM, Ling A, Kelly SJ, Kleiner DE, Mullikin JC, Ganson NJ, Kong HH, Hambleton S, Candotti F, Quezado MM, Calvo KR, Alao H, Barham BK, Jones A, Meschia JF, Worrall BB, Kasner SE, Rich SS, Goldbach-Mansky R, Abinun M, Chalom E, Gotte AC, Punaro M, Pascual V, Verbsky JW, Torgerson TR, Singer NG, Gershon TR, Ozen S, Karadag O, Fleisher TA, Remmers EF, Burgess SM, Moir SL, Gadina M, Sood R, Hershfield MS, Boehm M, Kastner DL, Aksentijevich I (March 2014). "Early-onset stroke and vasculopathy associated with mutations in ADA2". N. Engl. J. Med. 370 (10): 911–20. doi:10.1056/NEJMoa1307361. PMC 4193683. PMID 24552284.
  7. Navon Elkan P, Pierce SB, Segel R, Walsh T, Barash J, Padeh S, Zlotogorski A, Berkun Y, Press JJ, Mukamel M, Voth I, Hashkes PJ, Harel L, Hoffer V, Ling E, Yalcinkaya F, Kasapcopur O, Lee MK, Klevit RE, Renbaum P, Weinberg-Shukron A, Sener EF, Schormair B, Zeligson S, Marek-Yagel D, Strom TM, Shohat M, Singer A, Rubinow A, Pras E, Winkelmann J, Tekin M, Anikster Y, King MC, Levy-Lahad E (March 2014). "Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy". N. Engl. J. Med. 370 (10): 921–31. doi:10.1056/NEJMoa1307362. PMID 24552285.
  8. Griffin JW (November 2001). "Vasculitic neuropathies". Rheum. Dis. Clin. North Am. 27 (4): 751–60, vi. PMID 11723762.