Skip to main content

Advertisement

SpringerLink
Log in

Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa

  • Vasculitis (C Dejaco and C Duftner, Section Editors)
  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Purpose of the Review

Polyarteritis nodosa is a rare disease characterized by the necrotizing inflammation of medium-sized arteries. Different etiopathogenetic and clinical variants of the disease have been recognized over the past decades. In the present paper, we review the clinical features, diagnosis, and treatment of the different subtypes of the disease.

Recent Findings

The diagnosis of polyarteritis nodosa is primarily based on clinical findings, imaging, and histopathological investigations. Microbiological and genetic investigations complement the diagnostic work-up. Idiopathic and hereditary variants of polyarteritis nodosa are treated with immunomodulatory medications such as glucocorticoids, conventional immunomodulatory drugs (e.g., cyclophosphamide) and biologic agents (e.g., tumor necrosis factor inhibitors, interleukin 6 inhibitor), while hepatitis B virus-associated polyarteritis nodosa primarily requires antiviral therapy combined with plasma exchange.

Summary

PAN is a disease with heterogeneous presentations, severity, and therapeutic approaches. The overall prognosis of this disease is improving, mainly due to early diagnosis and more effective treatments. Treatment choices are guided mainly by the disease subtype and severity. In this review, we have presented the current knowledge on PAN clinical variants, their classification, diagnosis, and treatment approaches.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

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

    Google Scholar 

  2. 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;37:187–92.

    Article  CAS  Google Scholar 

  3. Ferrari E. Über Polyarteritis acuta nodosa (sogenannte Periarteritis nodosa), und ihre Beziehungen zur Polymyositis und Polyneuritis acuta. Beitr Pathol Anat. 1903;34:350–86.

    Google Scholar 

  4. Lindberg K. Ein beitrag zur kenntnis der periarteritis nodosa. Acta Med Scand. 1931;76:183.

    Article  Google Scholar 

  5. Trepo C, Thivolet J. Hepatitis associated antigen and periarteritis nodosa (PAN). Vox Sang. 1970;19:410–1.

    Article  CAS  Google Scholar 

  6. Patel N, Patel N, Khan T, Patel N, Espinoza LR. HIV infection and clinical spectrum of associated vasculitides. Curr Rheumatol Rep. 2011;13:506–12. https://doi.org/10.1007/s11926-011-0214-6.

    Article  PubMed  Google Scholar 

  7. Teng GG, Chatham WW. Vasculitis related to viral and other microbial agents. Best Pract Res Clin Rheumatol. 2015;29(2):226–43. https://doi.org/10.1016/j.berh.2015.05.007.

    Article  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;65:1–11. https://doi.org/10.1002/art.37715.

    Article  CAS  PubMed  Google Scholar 

  9. Sunderkötter CH, Zelger B, Chen KR, Requena L, Piette W, Carlson JA, et al. Nomenclature of cutaneous vasculitis: dermatologic addendum to the 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheumatol. 2018;70:171–84. https://doi.org/10.1002/art.40375.

    Article  PubMed  Google Scholar 

  10. 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. https://doi.org/10.1016/j.semarthrit.2012.03.006.

    Article  CAS  PubMed  Google Scholar 

  11. Parker M, McGill NW. Minocycline-associated polyarteritis nodosa and self-limiting hepatitis: a reminder of a potentially re-emerging culprit in drug-induced autoimmune syndromes. Scand J Rheumatol. 2018;47(6):507–8. https://doi.org/10.1080/03009742.2017.1416669.

    Article  PubMed  Google Scholar 

  12. Hernández-Rodríguez J, Gary S, Hoffman GS. Updating single-organ vasculitis. Curr Opin Rheumatol. 2012;24(1):38–45. https://doi.org/10.1097/BOR.0b013e32834d8482.

    Article  PubMed  Google Scholar 

  13. Navon Elkan EP, Pierce SB, Segel R, Walsh T, Barash J, Padeh S, et al. Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy. N Engl J Med. 2014;370:921–31. https://doi.org/10.1056/NEJMoa1307362.

    Article  CAS  PubMed  Google Scholar 

  14. 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;370:911–20. https://doi.org/10.1056/NEJMoa1307361.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Özçakar ZB, Çakar N, Uncu N, Çelikel BA, Yalçinkaya F. Familial Mediterranean fever-associated diseases in children. QJM. 2017;110:287–90. https://doi.org/10.1093/qjmed/hcw230.

    Article  CAS  PubMed  Google Scholar 

  16. Tunca M, Akar S, Onen F, Ozdogan H, Kasapcopur O, Yalcinkaya F, et al. Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine. 2005;84:1–11. https://doi.org/10.1097/01.md.0000152370.84628.0c.

    Article  Google Scholar 

  17. 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;46:1329–37. https://doi.org/10.1093/rheumatology/kem107.

    Article  CAS  Google Scholar 

  18. Gonzalez-Gay MA, Garcia-Porrua C, Guerrero J, Rodriguez-Ledo P, Llorca J. The epidemiology of the primary systemic vasculitides in Northwest Spain: implications of the Chapel Hill consensus conference definitions. Arthritis Rheum. 2003;49(3):388–93. https://doi.org/10.1002/art.11115.

    Article  PubMed  Google Scholar 

  19. Watts RA, Lane SE, Bentham G, Scott DG. Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. Arthritis Rheum. 2000;43(2):414–9. https://doi.org/10.1002/1529-0131(200002)43:2<414::AID-ANR23>3.0.CO;2-0.

    Article  CAS  PubMed  Google Scholar 

  20. Watts RA, Lane SE, Scott DG, Koldingsnes W, Nossent H, Gonzalez-Gay MA, et al. Epidemiology of vasculitis in Europe. Ann Rheum Dis. 2001;60:1156e7–1157. https://doi.org/10.1136/ard.60.12.1156a.

    Article  Google Scholar 

  21. Trepo C, Guillevin L. Polyarteritis nodosa and extrahepatic manifestations of HBV infection: the case against autoimmune intervention in pathogenesis. J Autoimmun. 2001;16:269–74. https://doi.org/10.1006/jaut.2000.0502.

    Article  CAS  PubMed  Google Scholar 

  22. Karadag O, Erden A, Bilginer Y, Gopaluni S, Sari A, Armagan B. A retrospective study comparing the phenotype and outcomes of patients with polyarteritis nodosa between UK and Turkish cohorts. Rheumatol Int. 2018;38:1833–40. https://doi.org/10.1007/s00296-018-4122-1.

    Article  PubMed  Google Scholar 

  23. 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;62:616–26. https://doi.org/10.1002/art.27240.

    Article  PubMed  Google Scholar 

  24. Ozen S, Anton J, Arisoy N, Bakkaloglu A, Besbas N, Brogan P, et al. Juvenile polyarteritis: results of a multicenter survey of 110 children. J Pediatr. 2004;145:517–22. https://doi.org/10.1016/j.jpeds.2004.06.046.

    Article  PubMed  Google Scholar 

  25. Alibaz-Oner F, Koster MJ, Crowson CS, Makol A, Steven R, Ytterberg SR, et al. Clinical spectrum of medium-sized vessel vasculitis. Arthritis Care Res (Hoboken). 2017;69(6):884–91. https://doi.org/10.1002/acr.23007.

    Article  CAS  Google Scholar 

  26. Chasset F, Francès C. Cutaneous manifestations of medium- and large-vessel vasculitis. Clin Rev Allergy Immunol. 2017;53(3):452–68. https://doi.org/10.1007/s12016-017-8612-9.

    Article  CAS  PubMed  Google Scholar 

  27. de Boysson H, Guillevin L. Polyarteritis nodosa neurologic manifestations. Neurol Clin. 2019;37(2):345–57. https://doi.org/10.1016/j.ncl.2019.01.007.

    Article  PubMed  Google Scholar 

  28. Ebert EC, Hagspiel KD, Nagar M, Schlesinger N. Gastrointestinal involvement in polyarteritis nodosa. Clin Gastroenterol Hepatol. 2008;6:960–6. https://doi.org/10.1016/j.cgh.2008.04.004.

    Article  PubMed  Google Scholar 

  29. Maritati F, Iannuzzella F, Pavia MP, Pasquali S, Vaglio A. Kidney involvement in medium- and large-vessel vasculitis. J Nephrol. 2016;29:495–505. https://doi.org/10.1007/s40620-016-0303-8.

    Article  CAS  PubMed  Google Scholar 

  30. Miloslavsky E, Unizony S. The heart in vasculitis. Rheum Dis Clin N Am. 2014;40:11–26. https://doi.org/10.1016/j.rdc.2013.10.006.

    Article  Google Scholar 

  31. Bae YD, Choi HJ, Lee JC, Park JJ, Lee YJ, Lee EB, et al. Clinical features of polyarteritis nodosa in Korea. J Korean Med Sci. 2006;21:591–5. https://doi.org/10.3346/jkms.2006.21.4.591.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Rothschild PR, Pagnoux C, Seror R, Brézin AP, Delair E, Guillevin L. Ophthalmologic manifestations of systemic necrotizing vasculitides at diagnosis: a retrospective study of 1286 patients and review of the literature. Semin Arthritis Rheum. 2013;42:507–14. https://doi.org/10.1016/j.semarthrit.2012.08.003.

    Article  PubMed  Google Scholar 

  33. Ungprasert P, Koster MJ, Thongprayoon C, Warrington KJ. Risk of venous thromboembolism among patients with vasculitis: a systematic review and meta-analysis. Clin Rheumatol. 2016;35:2741–7. https://doi.org/10.1007/s10067-016-3394-7.

    Article  PubMed  Google Scholar 

  34. Frohent PP, Sheps SG. Long-term follow-up study of periarteritis nodosa. Am J Med. 1967;43:8–14. https://doi.org/10.1016/0002-9343(67)90144-1.

    Article  Google Scholar 

  35. Jardel S, Puéchal 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;17:653–9. https://doi.org/10.1016/j.autrev.2018.01.022.

    Article  PubMed  Google Scholar 

  36. Gayraud M, Guillevin L, Toumelin PL, Cohen P, Lhote F, Casassus P, et al. Long-term follow up of polyarteritis nodosa, microscopic polyangiitis and Churg Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum. 2001;44:666–75. https://doi.org/10.1002/1529-0131(200103)44:3<666::AID-ANR116>3.0.CO;2-A.

    Article  CAS  PubMed  Google Scholar 

  37. 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;75:17–28. https://doi.org/10.1097/00005792-199601000-00003.

    Article  CAS  Google Scholar 

  38. Guillevin L, Mahr A, Callard P, Godmer P, Pagnoux C, Leray E, et al. Hepatitis B virus-associated polyarteritis nodosa. Clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine. 2005;84:313–22. https://doi.org/10.1097/01.md.0000180792.80212.5e.

    Article  PubMed  Google Scholar 

  39. Criado PR, Marques GF, Morita TC, de Carvalho JF. Epidemiological, clinical and laboratory profiles of cutaneous polyarteritis nodosa patients: report of 22 cases and literature review. Autoimmun Rev. 2016;15:558–63. https://doi.org/10.1016/j.autrev.2016.02.010.

    Article  PubMed  Google Scholar 

  40. Morgan AJ, Schwartz RA. Cutaneous polyarteritis nodosa: a comprehensive review. Int J Dermatol. 2010;49:750–6. https://doi.org/10.1111/j.1365-4632.2010.04522.x.

    Article  PubMed  Google Scholar 

  41. Daoud MS, Hutton KP, Gibson LE. Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases. Br J Dermatol. 1997;136:706–13.

    Article  CAS  Google Scholar 

  42. Kato A, Hamada T, Miyake T, Morizane S, Hirai Y, Yamasaki O, et al. Clinical and laboratory markers associated with relapse in cutaneous polyarteritis nodosa. JAMA Dermatol. 2018;154(8):922–6. https://doi.org/10.1001/jamadermatol.2018.1601.

    Article  PubMed  PubMed Central  Google Scholar 

  43. Munera-Campos M, Bielsa I, Martínez-Morillo M, Aparicio G, Olivé A, Ferrándiz C. Manifestations, clinical course and prognostic markers in cutaneous polyarteritis nodosa. J Dtsch Dermatol Ges. 2020;18:1250–9. https://doi.org/10.1111/ddg.14271.

    Article  PubMed  Google Scholar 

  44. Chen KR. Cutaneous polyarteritis nodosa: a clinical and histopathological study of 20 cases. J Dermatol. 1989;16:429–42.

    Article  CAS  Google Scholar 

  45. Kendall JL, Michael J, Springer JM. The many faces of a monogenic autoinflammatory disease: adenosine deaminase 2 deficiency. Current Rheumatology Reports. 2020;22:64. https://doi.org/10.1007/s11926-020-00944-1.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Human A, Pagnoux C. Diagnosis and management of ADA2 deficient polyarteritis nodosa. Int J Rheum Dis. 2019;22(Suppl 1):69–77. https://doi.org/10.1111/1756-185X.13283.

    Article  CAS  PubMed  Google Scholar 

  47. Moens L, Hershfield M, Arts K, Aksentijevich I, Meyts I. Human adenosine deaminase 2 deficiency: a multi-faceted inborn error of immunity Immunol Rev. Jan. 2019;287:62–72. https://doi.org/10.1111/imr.12722.

    Article  CAS  Google Scholar 

  48. Sahin S, Adrovic A, Kasapcopur O. A monogenic autoinflammatory disease with fatal vasculitis: deficiency of adenosine deaminase 2. Curr Opin Rheumatol. 2020;32:3–14. https://doi.org/10.1097/BOR.0000000000000669.

    Article  CAS  PubMed  Google Scholar 

  49. Jayne D. The diagnosis of vasculitis. Best Pract Res Clin Rheumatol. 2009;23:445–53. https://doi.org/10.1016/j.berh.2009.03.001.

    Article  PubMed  Google Scholar 

  50. Sönmez HE, Armağan B, Ayan G, Barut K, Batu ED, Erden A, et al. Polyarteritis nodosa: lessons from 25 years of experience. Clin Exp Rheumatol. 2019;37(Suppl 117):52–6.

    PubMed  Google Scholar 

  51. Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. 2007;66:222e7–27. https://doi.org/10.1136/ard.2006.054593.

    Article  Google Scholar 

  52. Stanson AW, Friese JL, Johnson CM, McKusick MA, Breen JF, Sabater EA, et al. Polyarteritis nodosa: spectrum of angiographic findings. Radiographics. 2001;21:151–9. https://doi.org/10.1148/radiographics.21.1.g01ja16151.

    Article  CAS  PubMed  Google Scholar 

  53. Hekali P, Kajander H, Pajari R, Stenman S, Somer T. Diagnostic significance of angiographically observed visceral aneurysms with regard to polyarteritis nodosa. Acta Radiol. 1991;32:143–8.

    Article  CAS  Google Scholar 

  54. Ozaki K, Miyayama S, Ushiogi Y, Matsui O. Renal involvement of polyarteritis nodosa: CT and MR findings. Abdom Imaging. 2009;34:265–70. https://doi.org/10.1007/s00261-008-9377-7.

    Article  PubMed  Google Scholar 

  55. Bleeker-Rovers CP, Bredie SJ, van der Meer JW, Corstens FH, Oyen WJ. F-18-fluorodeoxyglucose positron emission tomography in diagnosis and follow-up of patients with different types of vasculitis. Neth J Med. 2003;61:323–9.

    CAS  PubMed  Google Scholar 

  56. Watanabe TT, Shiojiri T. PET-CT and polyarteritis nodosa-associated artery aneurysms. QJM. 2019;1(112):219–20. https://doi.org/10.1093/qjmed/hcy308.

    Article  Google Scholar 

  57. Wang H, Li J, Jiang Y, et al. Polyarteritis nodosa with multiple aneurysms and renal arteriovenous fistula successfully diagnosed by colour Doppler sonography. Clin Rheumatol. 2013;32(Suppl. 1):S89e92. https://doi.org/10.1007/s10067-010-1519-y.

    Article  Google Scholar 

  58. Soudack M, Gaitini D, Ofer A. Celiac artery aneurysm: diagnosis by color doppler sonography and three-dimensional CT angiography. J Clin Ultrasound. 1999;27:49–51. https://doi.org/10.1002/(sici)1097-0096(199901)27:1<49::aid-jcu9>3.0.co;2-z.

    Article  CAS  PubMed  Google Scholar 

  59. Darras-Joly C, Lortholary O, Cohen P, Brauner M, Guillevin L. Regressing microaneurysms in 5 cases of hepatitis B virus related polyarteritis nodosa. J Rheumatol. 1995;22:876–80.

    CAS  PubMed  Google Scholar 

  60. Albert DA, Rimon D, Silverstein MD. The diagnosis of polyarteritis nodosa. I A literature-based decision analysis approach. Arthritis Rheum. 1988;31:1117e27. https://doi.org/10.1002/art.1780310906.

    Article  Google Scholar 

  61. Weinrich JM, Lenz A, Adam G, François CJ, Bannas P. Radiologic imaging in large and medium vessel vasculitis. Radiol Clin N Am. 2020;58(4):765–79. https://doi.org/10.1016/j.rcl.2020.02.001.

    Article  PubMed  Google Scholar 

  62. Lie JT. Systemic and isolated vasculitis. A rational approach to classification and pathologic diagnosis. Pathol Annu. 1989;24:25–114.

    PubMed  Google Scholar 

  63. Cid MC, Grau JM, Casademont J, Campo E, Coll-Vinent B, López-Soto A, et al. Immunohistochemical characterization of inflammatory cells and immunology activation markers in muscle and nerve biopsy specimens from patients with systemic polyarteritis nodosa. Arthritis Rheum. 1994;37:1055–61. https://doi.org/10.1002/art.1780370711.

    Article  CAS  PubMed  Google Scholar 

  64. Holl-Ulrich K, Noack F, Feller AC. Vasculitis: histopathology and differential diagnosis. Z Rheumatol. 2009;68:320–8. https://doi.org/10.1007/s00393-008-0402-6.

    Article  CAS  PubMed  Google Scholar 

  65. Zavialov AV, Garcia E, Glaichenhaus N, Franco R, Zavialov AV, Lauvau G. Human adenosine deaminase 2 induces differentiation of monocytes into macrophages and stimulates proliferation of T helper cells and macrophages. J Leukoc Biol. 2010;88:279–90. https://doi.org/10.1189/jlb.1109764.

    Article  CAS  PubMed  Google Scholar 

  66. Zavialov AV, Engstrom A. Human ADA2 belongs to a new family of growth factors with adenosine deaminase activity. Biochem J. 2005;391:51–7. https://doi.org/10.1042/BJ20050683.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  67. Zavialov AV, Yu X, Spillmann D, Lauvau G, Zavialov AV. Structural basis for the growth factor activity of human adenosine deaminase ADA2. J Biol Chem. 2010;285:12367–77. https://doi.org/10.1074/jbc.M109.083527.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  68. Karmouty-Quintana H, Xia Y, Blackburn MR. Adenosine signaling during acute and chronic disease states. J Mol Med (Berl). 2013;91:173–81. https://doi.org/10.1007/s00109-013-0997-1.

    Article  CAS  Google Scholar 

  69. Fries JF, Hunder GG, Bloch DA, Michel BA, Arend WP, Calabrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary Arthritis Rheum. 1990 Aug;33(8):1135e6–1136. https://doi.org/10.1002/art.1780330812.

    Article  Google Scholar 

  70. Lightfoot RW Jr, Michel BA, Bloch DA, Hunder GG, Zvaifler NJ, Mc Shane DJ, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990;33(8):1088e93.

    Google Scholar 

  71. Yamamoto S, Oiwa H. Provisional seven-item criteria for the diagnosis of polyarteritis nodosa. Rheumatol Int. 2020;40(8):1223–7. https://doi.org/10.1007/s00296-020-04535-2.

    Article  PubMed  Google Scholar 

  72. Seeliger B, Sznajd J, Robson JC, Judge A, Craven A, Grayson PC, et al. Are the 1990 American College of Rheumatology vasculitis classification criteria still valid? Rheumatology (Oxford). 2017;56(7):1154–61. https://doi.org/10.1093/rheumatology/kex075.

    Article  CAS  PubMed Central  Google Scholar 

  73. Bloch DA, Michel BA, Hunder GG, McShane DJ, Arend WP, Calabrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Patients and methods. Arthritis Rheum. 1990;33(8):1068e73–1073. https://doi.org/10.1002/art.1780330803.

    Article  Google Scholar 

  74. Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010;69:798e806. https://doi.org/10.1136/ard.2009.116657.

    Article  Google Scholar 

  75. Luqmani RA, Suppiah R, Grayson PC, Merkel PA, Watts R. Nomenclature and classification of vasculitis– update on the ACR/EULAR diagnosis and classification of vasculitis study (DCVAS). Clin Exp Immunol. 2011;164(Suppl 1):11–3. https://doi.org/10.1111/j.1365-2249.2011.04358.x.

    Article  PubMed  PubMed Central  Google Scholar 

  76. Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Khalid S, et al. OP0021 draft classification criteria for the anca associated vasculitides. Ann Rheum Dis. 2018;77:60–1. https://doi.org/10.1136/annrheumdis-2018-eular.2892.

    Article  Google Scholar 

  77. Ponte C, Grayson P, Suppiah R, Robson J, Gribbons K, Craven A, et al. 077. Classification criteria for large-vessel vasculitis, Rheumatology, Volume 58, Supplement 2, March 2019, kez058.017, https://doi.org/10.1093/rheumatology/kez058.017

  78. Henegar C, Pagnoux C, Puéchal X, Zucker JD, Bar-Hen A, Le Guern V, et al. A paradigm of diagnostic criteria for polyarteritis nodosa: analysis of a series of 949 patients with vasculitides. Arthritis Rheum. 2008;58(5):1528–38. https://doi.org/10.1002/art.23470.

    Article  PubMed  Google Scholar 

  79. Mukhtyar C, Guillevin L, Cid MC, , Dasgupta D, 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):310–317. https://doi.org/10.1136/ard.2008.088096.

    Article  CAS  PubMed  Google Scholar 

  80. Guillevin L, Cohen P, Mahr A, Arène JP, Mouthon L, Puéchal 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. https://doi.org/10.1002/art.10922.

    Article  CAS  PubMed  Google Scholar 

  81. Ribi C, Cohen P, Pagnoux C, Mahr A, Arène JP, Puéchal X, et al. French Vasculitis study group. 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. https://doi.org/10.1002/art.27340.

    Article  PubMed  Google Scholar 

  82. Puechal X, Pagnoux C, Baron G, Quemeneur T, Neel A, Agard C. Adding azathioprine to remission-induction glucocorticoids for eosinophilic granulomatosis with polyangiitis (Churg-Strauss), microscopic polyangiitis, or polyarteritis nodosa without poor prognosis factors. Arthritis Rheumatol. 2017;69(11):2175–86. https://doi.org/10.1002/art.40205.

    Article  CAS  PubMed  Google Scholar 

  83. Samson M, Puéchal X, Devilliers H, Ribi C, Cohen P, Bienvenu B, et al. Mononeuritis multiplex predicts the need for immunosuppressive or immunomodulatory drugs for EGPA, PAN and MPA patients without poor prognosis factors. Autoimmun Rev. 2014;13(9):945–53. https://doi.org/10.1016/j.autrev.2014.08.002.

    Article  CAS  PubMed  Google Scholar 

  84. 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. https://doi.org/10.1093/rheumatology/36.12.1290.

    Article  CAS  PubMed  Google Scholar 

  85. de Menthon M, Mahr A. Treating polyarteritis nodosa: current state of the art. Clin Exp Rheumatol. 2011;29(1 Suppl 64):S110–6.

    PubMed  Google Scholar 

  86. Muratore F, Pazzola G, Soriano A, Pipitone N, Croci S, Bonacini M, et al. Unmet needs in the pathogenesis and treatment of vasculitides. Clin Rev Allergy Immunol. 2018;54:244–60. https://doi.org/10.1007/s12016-017-8643-2.

    Article  CAS  PubMed  Google Scholar 

  87. 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(10):2825–33. https://doi.org/10.1007/s10067-019-04474-9.

    Article  PubMed  Google Scholar 

  88. Seri Y, Shoda H, Hanata N, Nagafuchi Y, Sumitomo S, Fujio K, et al. A case of refractory polyarteritis nodosa successfully treated with rituximab. Mod Rheumatol. 2017;27(4):696–8. https://doi.org/10.3109/14397595.2015.1014153.

    Article  PubMed  Google Scholar 

  89. Ostrovršnik J, Hočevar A, Lestan B, Sodin Semrl S, Lakota K, Tomsic M. Long-term follow-up on tocilizumab treatment of AA amyloidosis secondary to polyarteritis nodosa. Amyloid. 2016;23:260–1. https://doi.org/10.1080/13506129.2016.1232648.

    Article  PubMed  Google Scholar 

  90. Krusche M, Ruffer N, Kötter I. Tocilizumab treatment in refractory polyarteritis nodosa: a case report and review of the literature. Rheumatol Int. 2019;39(2):337–44. https://doi.org/10.1007/s00296-018-4210-2.

    Article  CAS  PubMed  Google Scholar 

  91. Krusche M, Ruffer N, Schneider U, Meyer M, Burmester G, Kötter I. Tocilizumab treatment for polyarteritis nodosa. Rheumatology (Oxford). 2020;59(10):e63–5. https://doi.org/10.1093/rheumatology/keaa079.

    Article  Google Scholar 

  92. Akiyama M, Kaneko Y, Takeuchi T. Tocilizumab for the treatment of polyarteritis nodosa: a systematic literature review. Ann Rheum Dis. 2020;9:annrheumdis-2020-218710. https://doi.org/10.1136/annrheumdis-2020-218710.

    Article  Google Scholar 

  93. Rimar D, Alpert A, Starosvetsky E, Rosner I, Slobodin G, Rozenbaum M, et al. Tofacitinib for polyarteritis nodosa: a tailored therapy. Ann Rheum Dis. 2016;75(12):2214–6. https://doi.org/10.1136/annrheumdis-2016-209330.

    Article  PubMed  Google Scholar 

  94. Michalak T. Immune complexes of hepatitis B surface antigen in the pathogenesis of periarteritis nodosa. Am J Pathol. 1978;90:619–32.

    CAS  PubMed  PubMed Central  Google Scholar 

  95. 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;20:289–98.

    CAS  PubMed  Google Scholar 

  96. 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;53:334–7. https://doi.org/10.1136/ard.53.5.334.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  97. 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. https://doi.org/10.1002/art.20401.

    Article  CAS  PubMed  Google Scholar 

  98. Caorsi R, Penco F, Grossi A, Insalaco A, Omenetti A, Alessio M, et al. ADA2 deficiency (DADA2) as an unrecognised cause of early onset polyarteritis nodosa and stroke: a multicentre national study. Ann Rheum Dis. 2017;76(10):1648–56. https://doi.org/10.1136/annrheumdis-2016-210802.

    Article  CAS  PubMed  Google Scholar 

  99. 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. https://doi.org/10.1056/NEJMc1801927.

    Article  PubMed  PubMed Central  Google Scholar 

  100. Hashem H, Kumar AR, Müller I, Babor F, Bredius R, Dalal J, et al. Hematopoietic stem cell transplantation rescues the hematological, immunological and vascular phenotype in DADA2. Blood. 2017;130:2682–8. https://doi.org/10.1182/blood-2017-07-798660.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  101. Van Eyck L Jr, Hershfield MS, Pombal D, Kelly SJ, Ganson NJ, Moens L, et al. Hematopoietic stem cell transplantation rescues the immunologic phenotype and prevents vasculopathy in patients with adenosine deaminase deficiency. J Allergy Clin Immunol. 2015;135(1):283–7. e5. https://doi.org/10.1016/j.jaci.2014.10.010.

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgments

The authors would like to thank Assoc. Prof. Vesna Jurčić, MD PhD and Assoc. Prof. Miroslav Mayer, MD PhD who kindly provided imaging material for the manuscript.

Funding

Alojzija Hočevar, Matija Tomšič and Katja Perdan Pirkmajer are supported by Slovenian Research Agency Grants P3-0314. Katja Perdan Pirkmajer is also supported by Slovenian Research Agency Grant J7-8276.

Author information

Authors and Affiliations

  1. Department of Rheumatology, University Medical Centre Ljubljana, Vodnikova cesta, 62 1000, Ljubljana, Slovenia

    Alojzija Hočevar, Matija Tomšič & Katja Perdan Pirkmajer

  2. Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia

    Alojzija Hočevar, Matija Tomšič & Katja Perdan Pirkmajer

Authors
  1. Alojzija Hočevar
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Matija Tomšič
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Katja Perdan Pirkmajer
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Alojzija Hočevar.

Ethics declarations

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Ethics Approval

Ethics approval was not needed.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical Collection on Vasculitis

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hočevar, A., Tomšič, M. & Perdan Pirkmajer, K. Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa. Curr Rheumatol Rep 23, 14 (2021). https://doi.org/10.1007/s11926-021-00983-2

Download citation

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s11926-021-00983-2

Keywords

Search

Navigation