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Systemic Polyarteritis Nodosa

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Large and Medium Size Vessel and Single Organ Vasculitis

Part of the book series: Rare Diseases of the Immune System ((RDIS))

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Abstract

Polyarteritis nodosa (PAN) is a rare systemic necrotizing vasculitis predominantly affecting the medium-sized arteries with widely variable presenting features, disease course, and outcomes. Recent updates regarding the nomenclature of PAN have resulted in the description of several PAN sub-phenotypes. Herein discussed are idiopathic PAN, Hepatitis B-associated PAN and monogenic disorders such as adenosine deaminase-2 deficiency. The current understanding of the pathogenesis, histopathological features, and treatment of these conditions are reviewed.

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References

  1. Kussmaul A, Maier K. Ueber eine nicht bisher beschriebene eigenhümliche Arterienerkrankung (Periarteritis Nodosa), die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskellähmung einhergeht. Dtsch Arch Klin Med. 1866;1:484–518.

    Google Scholar 

  2. Ferrari E. Uber polyarteritis acuta nodosa (sogenannte periarteritis nodosa) und ihre Beziehungen zur Polymyositis und Polyneuritis acuta. Zieglers Beitr. 1930;34:350–86.

    Google Scholar 

  3. Wegener F. Über eine eigenartige rhinogene Granulomatose mit besonderer Beteilgung des Arteriensystems und der Nieren. Beitr Path Anat. 1939;102:36–8.

    Google Scholar 

  4. Godman GC, Churg J. Wegener’s granulomatosis: pathology and review of the literature. AMA Arch Pathol. 1954;58(6):533–53.

    CAS  PubMed  Google Scholar 

  5. Davies DJ, Moran JE, Niall JF, Ryan GB. Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology? Br Med J (Clin Res Ed). 1982;285(6342):606.

    Article  CAS  Google Scholar 

  6. van der Woude FJ, Rasmussen N, Lobatto S, Wiik A, Permin H, van Es LA, et al. Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener’s granulomatosis. Lancet. 1985;1(8426):425–9.

    Article  PubMed  Google Scholar 

  7. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37(2):187–92.

    Article  CAS  PubMed  Google Scholar 

  8. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11.

    Article  CAS  PubMed  Google Scholar 

  9. Watts RA, Lane SE, Scott DG, Koldingsnes W, Nossent H, Gonzalez-Gay MA, et al. Epidemiology of vasculitis in Europe. Ann Rheum Dis. 2001;60(12):1156–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Mahr A, Guillevin L, Poissonnet M, Ayme S. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener’s granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum. 2004;51(1):92–9.

    Article  PubMed  Google Scholar 

  11. Mohammad AJ, Jacobsson LT, 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 (Oxford). 2007;46(8):1329–37.

    Article  CAS  Google Scholar 

  12. McMahon BJ, Bender TR, Templin DW, Maynard JE, Barrett DH, Berquist KR, et al. Vasculitis in Eskimos living in an area hyperendemic for hepatitis B. JAMA. 1980;244(19):2180–2.

    Article  CAS  PubMed  Google Scholar 

  13. Ozen S. The changing face of polyarteritis nodosa and necrotizing vasculitis. Nat Rev Rheumatol. 2017;13(6):381–6.

    Article  PubMed  Google Scholar 

  14. Cid MC, Grau JM, Casademont J, Campo E, Coll-Vinent B, Lopez-Soto A, et al. Immunohistochemical characterization of inflammatory cells and immunologic activation markers in muscle and nerve biopsy specimens from patients with systemic polyarteritis nodosa. Arthritis Rheum. 1994;37(7):1055–61.

    Article  CAS  PubMed  Google Scholar 

  15. Saadoun D, Terrier B, Semoun O, Sene D, Maisonobe T, Musset L, et al. Hepatitis C virus-associated polyarteritis nodosa. Arthritis Care Res (Hoboken). 2011;63(3):427–35.

    CAS  Google Scholar 

  16. Font C, Miro O, Pedrol E, Masanes F, Coll-Vinent B, Casademont J, et al. Polyarteritis nodosa in human immunodeficiency virus infection: report of four cases and review of the literature. Br J Rheumatol. 1996;35(8):796–9.

    Article  CAS  PubMed  Google Scholar 

  17. Corman LC, Dolson DJ. Polyarteritis nodosa and parvovirus B19 infection. Lancet. 1992;339(8791):491.

    Article  CAS  PubMed  Google Scholar 

  18. Caldeira T, Meireles C, Cunha F, Valbuena C, Aparicio J, Ribeiro A. Systemic polyarteritis nodosa associated with acute Epstein-Barr virus infection. Clin Rheumatol. 2007;26(10):1733–5.

    Article  PubMed  Google Scholar 

  19. Kouchi M, Sato S, Kamono M, Taoda A, Iijima K, Mizuma A, et al. A case of polyarteritis nodosa associated with cytomegalovirus infection. Case Rep Rheumatol. 2014;2014:604874.

    PubMed  PubMed Central  Google Scholar 

  20. Trepo C, Guillevin L. Polyarteritis nodosa and extrahepatic manifestations of HBV infection: the case against autoimmune intervention in pathogenesis. J Autoimmun. 2001;16(3):269–74.

    Article  CAS  PubMed  Google Scholar 

  21. Guillevin L, Ronco P, Verroust P. Circulating immune complexes in systemic necrotizing vasculitis of the polyarteritis nodosa group. Comparison of HBV-related polyarteritis nodosa and Churg Strauss Angiitis. J Autoimmun. 1990;3(6):789–92.

    Article  CAS  PubMed  Google Scholar 

  22. Michalak T. Immune complexes of hepatitis B surface antigen in the pathogenesis of periarteritis nodosa. A study of seven necropsy cases. Am J Pathol. 1978;90(3):619–32.

    CAS  PubMed  PubMed Central  Google Scholar 

  23. Katada Y, Harada Y, Azuma N, Matsumoto K, Terada H, Kudo E, et al. Minocycline-induced vasculitis fulfilling the criteria of polyarteritis nodosa. Mod Rheumatol. 2006;16(4):256–9.

    Article  PubMed  Google Scholar 

  24. Kermani TA, Ham EK, Camilleri MJ, Warrington KJ. Polyarteritis nodosa-like vasculitis in association with minocycline use: a single-center case series. Semin Arthritis Rheum. 2012;42(2):213–21.

    Article  CAS  PubMed  Google Scholar 

  25. Zhou Q, Yang D, Ombrello AK, Zavialov AV, Toro C, Zavialov AV, et al. Early-onset stroke and vasculopathy associated with mutations in ADA2. N Engl J Med. 2014;370(10):911–20.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Fayand A, Sarrabay G, Belot A, Hentgen V, Kone-Paut I, Grateau G, et al. [Multiple facets of ADA2 deficiency: vasculitis, auto-inflammatory disease and immunodeficiency: a literature review of 135 cases from literature]. Rev Med Interne. 2018;39(4):297–306.

    Google Scholar 

  27. Schnappauf O, Stoffels M, Aksentijevich I, Kastner D, Grayson P, Cuthbertson D, et al. Screening of patients with adult-onset idiopathic polyarteritis nodosa for deficiency of adenosine deaminase 2. Arthritis Rheumatol. 2018;70.

    Google Scholar 

  28. 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(2):616–26.

    Article  PubMed  Google Scholar 

  29. Alibaz-Oner F, Koster MJ, Crowson CS, Makol A, Ytterberg SR, Salvarani C, et al. Clinical spectrum of medium-sized vessel vasculitis. Arthritis Care Res (Hoboken). 2017;69(6):884–91.

    Article  CAS  Google Scholar 

  30. Lhote F, Cohen P, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis and Churg-Strauss syndrome. Lupus. 1998;7(4):238–58.

    Article  CAS  PubMed  Google Scholar 

  31. Merlin E, Mouy R, Pereira B, Mouthon L, Bourmaud A, Piette JC, et al. Long-term outcome of children with pediatric-onset cutaneous and visceral polyarteritis nodosa. Joint Bone Spine. 2015;82(4):251–7.

    Article  PubMed  Google Scholar 

  32. Minagar A, Fowler M, Harris MK, Jaffe SL. Neurologic presentations of systemic vasculitides. Neurol Clin. 2010;28(1):171–84.

    Article  PubMed  Google Scholar 

  33. de Boysson H, Guillevin L. Polyarteritis nodosa neurologic manifestations. Neurol Clin. 2019;37(2):345–57.

    Article  PubMed  Google Scholar 

  34. Levine SM, Hellmann DB, Stone JH. Gastrointestinal involvement in polyarteritis nodosa (1986-2000): presentation and outcomes in 24 patients. Am J Med. 2002;112(5):386–91.

    Article  PubMed  Google Scholar 

  35. Schrader ML, Hochman JS, Bulkley BH. The heart in polyarteritis nodosa: a clinicopathologic study. Am Heart J. 1985;109(6):1353–9.

    Article  CAS  PubMed  Google Scholar 

  36. Pagnoux C, Guillevin L. Cardiac involvement in small and medium-sized vessel vasculitides. Lupus. 2005;14(9):718–22.

    Article  CAS  PubMed  Google Scholar 

  37. Ebersberger U, Rieber J, Wellmann P, Goebel C, Gansera B. Polyarteritis nodosa causing a vast coronary artery aneurysm. J Am Coll Cardiol. 2015;65(5):e1–2.

    Article  PubMed  Google Scholar 

  38. Wi J, Choi HH, Lee CJ, Kim T, Shin S, Ko YG, et al. Acute myocardial infarction due to polyarteritis nodosa in a young female patient. Korean Circ J. 2010;40(4):197–200.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Calvo R, Negri M, Ortiz A, Roverano S, Paira S. Myositis as the initial presentation of panarteritis nodosa. Reumatol Clin. 2019;15(5):e24–6.

    Article  PubMed  Google Scholar 

  40. Kang Y, Hong SH, Yoo HJ, Choi JY, Park JK, Park J, et al. Muscle involvement in polyarteritis nodosa: report of eight cases with characteristic contrast enhancement pattern on MRI. AJR Am J Roentgenol. 2016;206(2):378–84.

    Article  PubMed  Google Scholar 

  41. Lie JT. Systemic and isolated vasculitis. A rational approach to classification and pathologic diagnosis. Pathol Annu. 1989;24(Pt 1):25–114.

    PubMed  Google Scholar 

  42. Jennette JC. Implications for pathogenesis of patterns of injury in small- and medium-sized-vessel vasculitis. Cleve Clin J Med. 2002;69(Suppl 2):SII33–8.

    PubMed  Google Scholar 

  43. Lightfoot RW Jr, Michel BA, Bloch DA, Hunder GG, Zvaifler NJ, McShane DJ, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990;33(8):1088–93.

    Article  PubMed  Google Scholar 

  44. Craven A, Robson J, Ponte C, Grayson PC, Suppiah R, Judge A, et al. ACR/EULAR-endorsed study to develop Diagnostic and Classification Criteria for Vasculitis (DCVAS). Clin Exp Nephrol. 2013;17(5):619–21.

    Article  CAS  PubMed  Google Scholar 

  45. Hernandez-Rodriguez J, Alba MA, Prieto-Gonzalez S, Cid MC. Diagnosis and classification of polyarteritis nodosa. J Autoimmun. 2014;48–49:84–9.

    Article  PubMed  CAS  Google Scholar 

  46. Jee KN, Ha HK, Lee IJ, Kim JK, Sung KB, Cho KS, et al. Radiologic findings of abdominal polyarteritis nodosa. AJR Am J Roentgenol. 2000;174(6):1675–9.

    Article  CAS  PubMed  Google Scholar 

  47. Singhal M, Gupta P, Sharma A, Lal A, Rathi M, Khandelwal N. Role of multidetector abdominal CT in the evaluation of abnormalities in polyarteritis nodosa. Clin Radiol. 2016;71(3):222–7.

    Article  CAS  PubMed  Google Scholar 

  48. Frohnert PP, Sheps SG. Long-term follow-up study of periarteritis nodosa. Am J Med. 1967;43(1):8–14.

    Article  CAS  PubMed  Google Scholar 

  49. Ribi C, Cohen P, Pagnoux C, Mahr A, Arene JP, Puechal X, et al. Treatment of polyarteritis nodosa and microscopic polyangiitis without poor-prognosis factors: a prospective randomized study of one hundred twenty-four patients. Arthritis Rheum. 2010;62(4):1186–97.

    Article  PubMed  Google Scholar 

  50. Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996;75(1):17–28.

    Article  CAS  Google Scholar 

  51. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P, et al. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011;90(1):19–27.

    Article  Google Scholar 

  52. Bourgarit A, Le Toumelin P, Pagnoux C, Cohen P, Mahr A, Le Guern V, et al. Deaths occurring during the first year after treatment onset for polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: a retrospective analysis of causes and factors predictive of mortality based on 595 patients. Medicine (Baltimore). 2005;84(5):323–30.

    Article  Google Scholar 

  53. Jardel S, Puechal X, Le Quellec A, Pagnoux C, Hamidou M, Maurier F, et al. Mortality in systemic necrotizing vasculitides: a retrospective analysis of the French Vasculitis Study Group registry. Autoimmun Rev. 2018;17(7):653–9.

    Article  PubMed  Google Scholar 

  54. Erden A, Batu ED, Sonmez HE, Sari A, Armagan B, Arici ZS, et al. Comparing polyarteritis nodosa in children and adults: a single center study. Int J Rheum Dis. 2017;20(8):1016–22.

    Article  CAS  PubMed  Google Scholar 

  55. Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009;68(3):310–7.

    Article  CAS  PubMed  Google Scholar 

  56. Pagnoux C, Quemeneur T, Ninet J, Diot E, Kyndt X, de Wazieres B, et al. Treatment of systemic necrotizing vasculitides in patients aged sixty-five years or older: results of a multicenter, open-label, randomized controlled trial of corticosteroid and cyclophosphamide-based induction therapy. Arthritis Rheumatol. 2015;67(4):1117–27.

    Article  CAS  PubMed  Google Scholar 

  57. Puechal X, Pagnoux C, Baron G, Quemeneur T, Neel A, Agard C, 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):2175–86.

    Article  CAS  PubMed  Google Scholar 

  58. Guillevin L, Cohen P, Mahr A, Arene JP, Mouthon L, Puechal X, et al. Treatment of polyarteritis nodosa and microscopic polyangiitis with poor prognosis factors: a prospective trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in sixty-five patients. Arthritis Rheum. 2003;49(1):93–100.

    Article  CAS  PubMed  Google Scholar 

  59. Gayraud M, Guillevin L, Cohen P, Lhote F, Cacoub P, Deblois P, et al. Treatment of good-prognosis polyarteritis nodosa and Churg-Strauss syndrome: comparison of steroids and oral or pulse cyclophosphamide in 25 patients. French Cooperative Study Group for Vasculitides. Br J Rheumatol. 1997;36(12):1290–7.

    Article  CAS  PubMed  Google Scholar 

  60. Eleftheriou D, Dillon MJ, Tullus K, Marks SD, Pilkington CA, Roebuck DJ, et al. Systemic polyarteritis nodosa in the young: a single-center experience over thirty-two years. Arthritis Rheum. 2013;65(9):2476–85.

    Article  CAS  PubMed  Google Scholar 

  61. Ribeiro E, Cressend T, Duffau P, Grenouillet-Delacre M, Rouanet-Lariviere M, Vital A, et al. Rituximab efficacy during a refractory polyarteritis nodosa flare. Case Rep Med. 2009;2009:738293.

    Article  PubMed  Google Scholar 

  62. Neel A, Masseau A, Hervier B, Bossard C, Cacoub P, Pagnoux C, et al. Life-threatening hepatitis C virus-associated polyarteritis nodosa successfully treated by rituximab. J Clin Rheumatol. 2011;17(8):439–41.

    Article  PubMed  Google Scholar 

  63. Krusche M, Ruffer N, Kotter I. Tocilizumab treatment in refractory polyarteritis nodosa: a case report and review of the literature. Rheumatol Int. 2019;39(2):337–44.

    Article  CAS  PubMed  Google Scholar 

  64. Capuozzo M, Ottaiano A, Nava E, Cascone S, Fico R, Iaffaioli RV, et al. Etanercept induces remission of polyarteritis nodosa: a case report. Front Pharmacol. 2014;5:122.

    Article  PubMed  PubMed Central  Google Scholar 

  65. Wang CR, Yang CC. Adalimumab therapy in hepatitis B virus-negative polyarteritis nodosa: a case report. Medicine (Baltimore). 2018;97(25):e11053.

    Article  Google Scholar 

  66. Lerkvaleekul B, Treepongkaruna S, Ruangwattanapaisarn N, Treesit T, Vilaiyuk S. Recurrent ruptured abdominal aneurysms in polyarteritis nodosa successfully treated with infliximab. Biologics. 2019;13:111–6.

    PubMed  PubMed Central  Google Scholar 

  67. Ginsberg S, Rosner I, Slobodin G, Rozenbaum M, Kaly L, Jiries N, et al. Infliximab for the treatment of refractory polyarteritis nodosa. Clin Rheumatol. 2019;38:2825.

    Article  PubMed  Google Scholar 

  68. Ombrello AK, Qin J, Hoffmann PM, Kumar P, Stone D, Jones A, et al. Treatment strategies for deficiency of adenosine deaminase 2. N Engl J Med. 2019;380(16):1582–4.

    Article  PubMed  PubMed Central  Google Scholar 

  69. Guillevin L, Mahr A, Cohen P, Larroche C, Queyrel V, Loustaud-Ratti V, et al. Short-term corticosteroids then lamivudine and plasma exchanges to treat hepatitis B virus-related polyarteritis nodosa. Arthritis Rheum. 2004;51(3):482–7.

    Article  CAS  PubMed  Google Scholar 

  70. Guillevin L, Lhote F, Leon A, Fauvelle F, Vivitski L, Trepo C. Treatment of polyarteritis nodosa related to hepatitis B virus with short term steroid therapy associated with antiviral agents and plasma exchanges. A prospective trial in 33 patients. J Rheumatol. 1993;20(2):289–98.

    CAS  PubMed  Google Scholar 

  71. Guillevin L, Lhote F, Cohen P, Jarrousse B, Lortholary O, Genereau T, et al. Corticosteroids plus pulse cyclophosphamide and plasma exchanges versus corticosteroids plus pulse cyclophosphamide alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome patients with factors predicting poor prognosis. A prospective, randomized trial in sixty-two patients. Arthritis Rheum. 1995;38(11):1638–45.

    Article  CAS  PubMed  Google Scholar 

  72. Guillevin L, Lhote F, Sauvaget F, Deblois P, Rossi F, Levallois D, et al. Treatment of polyarteritis nodosa related to hepatitis B virus with interferon-alpha and plasma exchanges. Ann Rheum Dis. 1994;53(5):334–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  73. Lok AS, McMahon BJ, Brown RS Jr, Wong JB, Ahmed AT, Farah W, et al. Antiviral therapy for chronic hepatitis B viral infection in adults: a systematic review and meta-analysis. Hepatology. 2016;63(1):284–306.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of Rheumatology, Department of Internal Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN, USA

    Matthew J. Koster

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  1. Rheumatology Unit, Azienda Unita’ Sanitaria Locale IRCCS di Reggio Emilia, Reggio Emilia, Italy

    Carlo Salvarani

  2. Rheumatology Unit, Azienda Unita’ Sanitaria Locale IRCCS di Reggio Emilia, Reggio Emilia, Italy

    Luigi Boiardi

  3. Rheumatology Unit, Azienda Unita’ Sanitaria Locale IRCCS di Reggio Emilia, Reggio Emilia, Italy

    Francesco Muratore

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Koster, M.J. (2021). Systemic Polyarteritis Nodosa. In: Salvarani, C., Boiardi, L., Muratore, F. (eds) Large and Medium Size Vessel and Single Organ Vasculitis. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-030-67175-4_14

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