Polyarteritis Nodosa Questions & Answers

1. Stanton M, Tiwari V. Polyarteritis Nodosa. 2022 Jan. [QxMD MEDLINE Link]. [Full Text].

2. Ozen S. The changing face of polyarteritis nodosa and necrotizing vasculitis. Nat Rev Rheumatol. 2017 Jun. 13 (6):381-386. [QxMD MEDLINE Link].

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

4. Forbess L, Bannykh S. Polyarteritis nodosa. Rheum Dis Clin North Am. 2015. 41 (1):33-46, vii. [QxMD MEDLINE Link].

5. Matteson EL. A history of early investigation in polyarteritis nodosa. Arthritis Care Res. 1999 Aug. 12(4):294-302. [QxMD MEDLINE Link].

6. Davson J, Ball J, Platt R. The kidney in periarteritis nodosa. Q J Med. 1948 Jul. 17(67):175-202. [QxMD MEDLINE Link].

7. Stone JH. Polyarteritis nodosa. JAMA. 2002 Oct 2. 288(13):1632-9. [QxMD MEDLINE Link].

8. Lightfoot RW Jr, Michel BA, Bloch DA, Hunder GG, Zvaifler NJ, McShane DJ. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990 Aug. 33(8):1088-93. [QxMD MEDLINE Link].

9. Jennette JC, Falk RJ, Andrassy K. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994 Feb. 37(2):187-92. [QxMD MEDLINE Link].

10. Colmegna I, Maldonado-Cocco JA. Polyarteritis nodosa revisited. Curr Rheumatol Rep. 2005 Aug. 7(4):288-96. [QxMD MEDLINE Link].

11. Trepo C, Guillevin L. Polyarteritis nodosa and extrahepatic manifestations of HBV infection: the case against autoimmune intervention in pathogenesis. J Autoimmun. 2001 May. 16(3):269-74. [QxMD MEDLINE Link].

12. Mahr A, Guillevin L, Poissonnet M, Aymé 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 Feb 15. 51(1):92-9. [QxMD MEDLINE Link].

13. Guillevin L, Lhote F, Cohen P, Sauvaget F, Jarrousse B, Lortholary O, et al. Polyarteritis nodosa related to hepatitis B virus. A prospective study with long-term observation of 41 patients. Medicine (Baltimore). 1995 Sep. 74(5):238-53. [QxMD MEDLINE Link].

14. Zhou Q, Yang D, Ombrello AK, Zavialov AV, Toro C, et al. Early-onset stroke and vasculopathy associated with mutations in ADA2. N Engl J Med. 2014 Mar 6. 370 (10):911-20. [QxMD MEDLINE Link].

15. Navon Elkan P, Pierce SB, Segel R, Walsh T, Barash J, et al. Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy. N Engl J Med. 2014 Mar 6. 370 (10):921-31. [QxMD MEDLINE Link]. [Full Text].

16. Gonzalez Santiago TM, Zavialov A, Saarela J, Seppanen M, Reed AM, Abraham RS, et al. Dermatologic Features of ADA2 Deficiency in Cutaneous Polyarteritis Nodosa. JAMA Dermatol. 2015 Nov 1. 151 (11):1230-4. [QxMD MEDLINE Link].

17. Caorsi R, Penco F, Grossi A, Insalaco A, Omenetti A, et al. ADA2 deficiency (DADA2) as an unrecognised cause of early onset polyarteritis nodosa and stroke: a multicentre national study. Ann Rheum Dis. 2017 May 18. [QxMD MEDLINE Link].

18. Soufir N, Descamps V, Crickx B. Hepatitis C virus infection in cutaneous polyarteritis nodosa: a retrospective study of 16 cases. Arch Dermatol. 1999 Aug. 135(8):1001-2. [QxMD MEDLINE Link].

19. Saadoun D, Terrier B, Semoun O, Sene D, Maisonobe T, Musset L, et al. Hepatitis C virus-associated polyarteritis nodosa. Arthritis Care Res (Hoboken). 2010 Oct 27. [QxMD MEDLINE Link].

20. Hughes LB, Bridges SL Jr. Polyarteritis nodosa and microscopic polyangiitis: etiologic and diagnostic considerations. Curr Rheumatol Rep. 2002 Feb. 4(1):75-82. [QxMD MEDLINE Link].

21. Somer T, Finegold SM. Vasculitides associated with infections, immunization, and antimicrobial drugs. Clin Infect Dis. 1995 Apr. 20(4):1010-36. [QxMD MEDLINE Link].

22. Patel N, Patel N, Khan T, Patel N, Espinoza LR. HIV infection and clinical spectrum of associated vasculitides. Curr Rheumatol Rep. 2011 Dec. 13(6):506-12. [QxMD MEDLINE Link].

23. Imanishi H, Tsuruta D, Oshimo T, Sowa J, Mizuno N, Nakagawa K, et al. Cutaneous polyarteritis nodosa induced by Mycobacterium tuberculosis. J Dermatol. 2012 Aug. 39(8):738-9. [QxMD MEDLINE Link].

24. Watts RA, Mooney J, Lane SE, Scott DG. Rheumatoid vasculitis: becoming extinct?. Rheumatology (Oxford). 2004 Jul. 43(7):920-3. [QxMD MEDLINE Link].

25. Nakano H, Ooka S, Shibata T, Ogawa H, Ito H, Takakuwa Y, et al. Cutaneous polyarteritis nodosa associated with HLA-B39-positive undifferentiated spondyloarthritis in a Japanese patient. Mod Rheumatol. 2012 Sep. 22(5):783-6. [QxMD MEDLINE Link].

26. Pagnini I, Simonini G, Lippi F, Azzari C, Cimaz R. Cutaneous polyarteritis nodosa and common variable immunodeficiency: a previously unreported association. Clin Exp Rheumatol. 2012 Jan-Feb. 30(1 Suppl 70):S169. [QxMD MEDLINE Link].

27. Oulego-Erroz I, Gautreaux-Minaya S, Martinez-Sáenz de Jubera J, Naranjo-Vivas D, Fernéndez-Hernández S, Muñíz-Fontán M. Coexistence of polyarteritis nodosa and psoriatic arthritis in a child: an unreported association: Polyarteritis nodosa and Psoriatic arthitritis. Eur J Pediatr. 2011 Sep. 170(9):1213-5. [QxMD MEDLINE Link].

28. Hasler P, Kistler H, Gerber H. Vasculitides in hairy cell leukemia. Semin Arthritis Rheum. 1995 Oct. 25(2):134-42. [QxMD MEDLINE Link].

29. Ambrosio MR, Rocca BJ, Ginori A, Onorati M, Fabbri A, Carmellini M, et al. Renal infarction due to polyarteritis nodosa in a patient with angioimmunoblastic T-cell lymphoma: a case report and a brief review of the literature. Diagn Pathol. 2012 May 8. 7:50. [QxMD MEDLINE Link]. [Full Text].

30. McMahon BJ, Heyward WL, Templin DW, Clement D, Lanier AP. Hepatitis B-associated polyarteritis nodosa in Alaskan Eskimos: clinical and epidemiologic features and long-term follow-up. Hepatology. 1989 Jan. 9(1):97-101. [QxMD MEDLINE Link].

31. Selga D, Mohammad A, Sturfelt G, Segelmark M. Polyarteritis nodosa when applying the Chapel Hill nomenclature--a descriptive study on ten patients. Rheumatology (Oxford). 2006 Oct. 45(10):1276-81. [QxMD MEDLINE Link].

32. Levine SM, Hellmann DB, Stone JH. Gastrointestinal involvement in polyarteritis nodosa (1986-2000): presentation and outcomes in 24 patients. Am J Med. 2002 Apr 1. 112(5):386-91. [QxMD MEDLINE Link].

33. 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 Aug 1. 154 (8):922-926. [QxMD MEDLINE Link].

34. Falcini F, La Torre F, Vittadello F, Rigante D, Martini G, Corona F, et al. Clinical overview and outcome in a cohort of children with polyarteritis nodosa. Clin Exp Rheumatol. 2014 May-Jun. 32(3 Suppl 82):S134-7. [QxMD MEDLINE Link].

35. 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 Sep. 65(9):2476-85. [QxMD MEDLINE Link].

36. Mahmood-Rao H, Ding T, Gandhi N. Gangrenous digital infarcts in a severe case of cutaneous polyarteritis nodosa. BMJ Case Rep. 2017 Jun 29. 2017:[QxMD MEDLINE Link].

37. Agarwal A, Bansal M, Pandey R, Swaminathan S. Bilateral subcapsular and perinephric hemorrhage as the initial presentation of polyarteritis nodosa. Intern Med. 2012. 51(9):1073-6. [QxMD MEDLINE Link].

38. Battula N, Tsapralis D, Morgan M, Mirza D. Spontaneous liver haemorrhage and haemobilia as initial presentation of undiagnosed polyarteritis nodosa. Ann R Coll Surg Engl. 2012 May. 94(4):e163-5. [QxMD MEDLINE Link].

39. Parent BA, Cho SW, Buck DG, Nalesnik MA, Gamblin TC. Spontaneous rupture of hepatic artery aneurysm associated with polyarteritis nodosa. Am Surg. 2010 Dec. 76(12):1416-9. [QxMD MEDLINE Link].

40. Yuce M, Davutoglu V, Sari I, Onat AM. Polyarteritis nodosa with multiple coronary aneurysms presenting as acute myocardial infarction. Am J Med Sci. 2011 May. 341(5):409. [QxMD MEDLINE Link].

41. Pagnoux C, Le Guern V, Goffinet F, Diot E, Limal N, Pannier E, et al. Pregnancies in systemic necrotizing vasculitides: report on 12 women and their 20 pregnancies. Rheumatology (Oxford). 2011 May. 50(5):953-61. [QxMD MEDLINE Link].

42. Kocyigit I, Koyuncu S, Mavili E, Unal A, Tokmak TT, Cilan H. Unusual clinic presentation of polyarteritis nodosa: involvement of axillary and brachial artery. Ren Fail. 2011. 33(10):1043-5. [QxMD MEDLINE Link].

43. Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996 Jan. 75(1):17-28. [QxMD MEDLINE Link].

44. Grinstein P, Maximiliano LS, Lucas Y, Norberto B. Polyarteritis nodosa with genital necrosis. Urol Case Rep. 2020 Nov. 33:101285. [QxMD MEDLINE Link]. [Full Text].

45. Fernandes SR, Coimbra IB, Costallat LT. Uncommon features of polyarteritis nodosa: psychosis and angio-oedema. Clin Rheumatol. 1998. 17(4):353-6. [QxMD MEDLINE Link].

46. Ganeshanandan LR, Brusch AM, Dyke JM, McLean-Tooke APC. Polyarteritis nodosa isolated to muscles-A case series with a review of the literature. Semin Arthritis Rheum. 2020 Jun. 50 (3):503-508. [QxMD MEDLINE Link].

47. Griffin JW. Vasculitic neuropathies. Rheum Dis Clin North Am. 2001 Nov. 27(4):751-60, vi. [QxMD MEDLINE Link].

48. Rosenberg MR, Parshley M, Gibson S, Wernick R. Central nervous system polyarteritis nodosa. West J Med. 1990 Nov. 153(5):553-6. [QxMD MEDLINE Link]. [Full Text].

49. Haroon M, Bermingham N, Keohane C, Harney S. Polyarteritis nodosa presenting with clinical and radiologic features suggestive of polymyositis. Rheumatol Int. 2012 Apr. 32(4):1079-81. [QxMD MEDLINE Link].

50. Yang SN, Cho NS, Choi HS, Choi SJ, Yoon ES, Kim DH. Muscular polyarteritis nodosa. J Clin Rheumatol. 2012 Aug. 18(5):249-52. [QxMD MEDLINE Link].

51. Filippone EJ, Foy A, Galanis T, Pokuah M, Newman E, Lallas CD. Segmental arterial mediolysis: report of 2 cases and review of the literature. Am J Kidney Dis. 2011 Dec. 58(6):981-7. [QxMD MEDLINE Link].

52. 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 Oct. 42(2):213-21. [QxMD MEDLINE Link].

53. Bezier M, Perceau G, Reguiai Z, Remy-Leroux V, Tchen T, Durlach A. [Necrotic leg ulcers induced by vitamin K antagonists: five cases]. Ann Dermatol Venereol. 2011 Oct. 138(10):657-63. [QxMD MEDLINE Link].

54. Higuchi T, Sugimoto N, Hayama M, Tanaka E. The usefulness of 3D-CT angiography in polyarteritis nodosa. Intern Med. 2012. 51(11):1449-50. [QxMD MEDLINE Link].

55. Özçakar ZB, Fitöz S, Yıldız AE, Yalçınkaya F. Childhood polyarteritis nodosa: diagnosis with non-invasive imaging techniques. Clin Rheumatol. 2017 Jan. 36 (1):165-171. [QxMD MEDLINE Link].

56. Hervier B, Durant C, Masseau A, Ponge T, Hamidou M, Mussini JM. Use of muscle biopsies for diagnosis of systemic vasculitides. J Rheumatol. 2011 Mar. 38(3):470-4. [QxMD MEDLINE Link].

57. Ricotti C, Kowalczyk JP, Ghersi M, Nousari CH. The diagnostic yield of histopathologic sampling techniques in PAN-associated cutaneous ulcers. Arch Dermatol. 2007 Oct. 143(10):1334-6. [QxMD MEDLINE Link].

58. Gheita TA, Khairy NA, Nasrallah MM, Hussein H. Subclinical renal involvement in essential cryoglobulinemic vasculitis and classic polyarteritis nodosa. Joint Bone Spine. 2012 May. 79(3):274-80. [QxMD MEDLINE Link].

59. Demirkaya E, Ozen S, Pistorio A, Galasso R, Ravelli A, Hasija R. Performance of Birmingham Vasculitis Activity Score and disease extent index in childhood vasculitides. Clin Exp Rheumatol. 2012 Jan-Feb. 30(1 Suppl 70):S162-8. [QxMD MEDLINE Link].

60. Campanilho-Marques R, Ramos F, Canhão H, Fonseca JE. Remission induced by infliximab in a childhood polyarteritis nodosa refractory to conventional immunosuppression and rituximab. Joint Bone Spine. 2014 May. 81 (3):277-8. [QxMD MEDLINE Link].

61. Matsuo S, Hayashi K, Morimoto E, Kato A, Sada KE, Watanabe H, et al. The Successful Treatment of Refractory Polyarteritis Nodosa Using Infliximab. Intern Med. 2017. 56 (11):1435-1438. [QxMD MEDLINE Link]. [Full Text].

62. Inoue N, Shimizu M, Mizuta M, Ikawa Y, Yachie A. Refractory cutaneous polyarteritis nodosa: Successful treatment with etanercept. Pediatr Int. 2017 Jun. 59 (6):751-752. [QxMD MEDLINE Link].

63. Akiyama M, Kaneko Y, Takeuchi T. Tocilizumab for the treatment of polyarteritis nodosa: a systematic literature review. Ann Rheum Dis. 2020 Sep 9. [QxMD MEDLINE Link].

64. Krusche M, Ruffer N, Kötter I. Tocilizumab treatment in refractory polyarteritis nodosa: a case report and review of the literature. Rheumatol Int. 2019 Feb. 39 (2):337-344. [QxMD MEDLINE Link].

65. Krusche M, Ruffer N, Schneider U, Meyer M, Burmester G, Kötter I. Tocilizumab treatment for polyarteritis nodosa. Rheumatology (Oxford). 2020 Oct 1. 59 (10):e63-e65. [QxMD MEDLINE Link].

66. 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. 2015 Mar 12. 1-3. [QxMD MEDLINE Link].

67. de Luna G, et al; French Vasculitis Study Group (FVSG). Plasma exchanges for the treatment of severe systemic necrotizing vasculitides in clinical daily practice: Data from the French Vasculitis Study Group. J Autoimmun. 2015 Dec. 65:49-55. [QxMD MEDLINE Link].

68. Frohnert PP, Sheps SG. Long-term follow-up study of periarteritis nodosa. Am J Med. 1967 Jul. 43(1):8-14. [QxMD MEDLINE Link].

69. Gayraud M, Guillevin L, le Toumelin P, Cohen P, Lhote F, Casassus P. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum. 2001 Mar. 44(3):666-75. [QxMD MEDLINE Link].

70. 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 Jun 15. 51(3):482-7. [QxMD MEDLINE Link].

71. 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 Feb. 20(2):289-98. [QxMD MEDLINE Link].

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

73. Erhardt A, Sagir A, Guillevin L, Neuen-Jacob E, Häussinger D. Successful treatment of hepatitis B virus associated polyarteritis nodosa with a combination of prednisolone, alpha-interferon and lamivudine. J Hepatol. 2000 Oct. 33(4):677-83. [QxMD MEDLINE Link].

74. Wicki J, Olivieri J, Pizzolato G, Sarasin F, Guillevin L, Dayer JM. Successful treatment of polyarteritis nodosa related to hepatitis B virus with a combination of lamivudine and interferon alpha. Rheumatology (Oxford). 1999 Feb. 38(2):183-5. [QxMD MEDLINE Link].

75. Molloy PJ, Friedlander L, Van Thiel DH. Combined interferon, famciclovir and GM-CSF treatment of HBV infection in an individual with periarteritis nodosa. Hepatogastroenterology. 1999 Jul-Aug. 46(28):2529-31. [QxMD MEDLINE Link].

76. de Menthon M, Mahr A. Treating polyarteritis nodosa: current state of the art. Clin Exp Rheumatol. 2011 Jan-Feb. 29(1 Suppl 64):S110-6. [QxMD MEDLINE Link].

77. Fernanda F, Serena C, Giustina R, Antonella B, Alessandra D, Alessio M. Mycophenolate mofetil treatment in two children with severe polyarteritis nodosa refractory to immunosuppressant drugs. Rheumatol Int. 2012 Jul. 32(7):2215-9. [QxMD MEDLINE Link].

78. Berlit P. Diagnosis and treatment of cerebral vasculitis. Ther Adv Neurol Disord. 2010 Jan. 3(1):29-42. [QxMD MEDLINE Link]. [Full Text].

79. Kawakami T, Soma Y. Use of mizoribine in two patients with recalcitrant cutaneous polyarteritis nodosa. J Am Acad Dermatol. 2011 Jun. 64(6):1213-4. [QxMD MEDLINE Link].

80. Kluger N, Guillot B, Bessis D. Ulcerative cutaneous polyarteritis nodosa treated with mycophenolate mofetil and pentoxifylline. J Dermatolog Treat. 2011 Jun. 22(3):175-7. [QxMD MEDLINE Link].

81. [Guideline] Chung SA, Gorelik M, Langford CA, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa. Arthritis Care Res (Hoboken). 2021 Aug. 73 (8):1061-1070. [QxMD MEDLINE Link]. [Full Text].

Dana Jacobs-Kosmin, MD, FACP Attending Physician, Department of Medicine, Division of Rheumatology, Einstein Medical Center; Clinical Assistant Professor of Medicine, Jefferson Medical College of Thomas Jefferson University

Dana Jacobs-Kosmin, MD, FACP is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Medical Association

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J Mark Jackson, MD Clinical Professor of Medicine/Dermatology, Division of Dermatology, University of Louisville School of Medicine

J Mark Jackson, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Kentucky Medical Association, Texas Medical Association

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Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

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Mahendra Agraharkar, MD, MBBS, FACP, FASN, Clinical Associate Professor of Medicine, Baylor College of Medicine; President and CEO, Space City Associates of Nephrology

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