Mini-review

The concise review of etiology, pathology, and treatment strategy of polyarteritis nodosa

Downloads

Ikeda, T. (2022). The concise review of etiology, pathology, and treatment strategy of polyarteritis nodosa. Trends in Immunotherapy, 6(2). https://doi.org/10.24294/ti.v6.i2.1452

Authors

  • Takaharu Ikeda
    Tohoku Medical and Pharmaceutical University

Polyarteritis nodosa (PAN) is a necrotizing vasculitis that targets medium-sized muscular arteries and can involve small-sized arteries. The pathogenesis of classic PAN remains unclear, except for secondary PAN or vasculitis that is indistinguishable from PAN due to genetic abnormalities such as deficiency of adenosine deaminase 2. The histopathological characters of PAN change over time from the onset. The type of remission induction therapy to be adopted depends on the disease severity. When it results in remission, corticosteroid dose reduction will begin and will be shifted to remission maintenance therapy.

Keywords:

Polyarteritis Nodosa; Deficiency of Adenosine Deaminase 2 Anti-Phosphatidylserine-Prothrombin Complex Antibodies

References

  1. Davies DJ, Moran JE, Niallnm JF, et al. Segmental necrotizing glomerulonephritis with antineutrophil antibody: Possible arbovirus etiology? British Medical Journal (Clinical Research Ed.) 1982; 285(6342): 606. doi: 10.1136/bmj.285.6342.606
  2. van der Woude FJ, Rasmussen N, Lobatto S, et al. Autoantibodies against neutrophils and monocytes: Tool for diagnosis and marker of disease activity in Wegener’s granulomatosis. The Lancet 1985; 325(8426): 425–429. doi: 10.1016/S0140-6736(85)91147-X
  3. Jennette JC, Falk RJ, Bacon PA, et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis & Rheumatism 2013; 65(1): 1–11. doi: 10.1002/art.37715
  4. Zhou Q, Dan Y, Ombrello AK, et al. Early-onset stroke and vasculopathy associated with mutations in ADA2. The New England Journal of Medicine 2014; 370: 911–920. doi: 10.1056/NEJMoa1307361
  5. Elkan PN, Pierce SB, Segal R, et al. Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy. The New England Journal of Medicine 2014; 370: 921–931. doi: 10.1056/NEJMoa1307362
  6. Barron KS, Aksentijevich I, Deuitch NT, et al. The spectrum of the deficiency of adenosine deaminase 2: An observational analysis of a 60 patient cohort. Frontiers in Immunology 2022; 12: 811473. doi: 10.3389/fimmu.2021.811473
  7. Gibson KM, Morishita KA, Dancey P, et al. Identification of novel adenosine deaminase 2 gene variants and varied clinical phenotype in pediatric vasculitis. Arthritis & Rheumatology 2019; 71(10): 1747–1755. doi: 10.1002/art.40913
  8. Chung SA, Gorelik M, Langford CA, et al. The 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of polyarteritis nodosa. Arthritis Care & Research 2021; 73(8): 1061–1070. doi: 10.1002/acr.24633
  9. Kawakami T, Yamazaki M, Mizoguchi M, et al. High titer of anti-phosphatidylserine-prothrombin complex antibodies in patients with cutaneous polyarteritis nodosa. Arthritis Care & Research 2007; 57(8): 1507–1513. doi: 10.1002/art.23081
  10. Okano T, Takeuchi S, Soma Y, et al. Presence of anti-phosphatidylserine-prothrombin complex antibodies and anti-moesin antibodies in patients with polyarteritis nodosa. The Journal of Dermatology 2017; 44(1): 18–22. doi: 10.1111/1346-8138.13491
  11. Kawakami T, Tamura Y, Dong Y, et al. Anti-phosphatidylserine/prothrombin complex antibodies in patients with cutaneous vasculitis: Possible involvement in the pathogenesis. The Journal of Dermatology 2021; 48(5): 703–706. doi: 10.1111/1346-8138.15810
  12. Ikeda T, Furukawa F, Kawakami T, et al. Outline of guidelines for the management of vasculitis and vascular disorders in Japan, 2016 revised edition. The Journal of Dermatology 2018; 45(2): 122–127. doi: 10.1111/1346-8138.14086
  13. Isobe M, Amano K, Arimura Y, et al. JCA 2017 guideline on management of vasculitis syndrome. Circulation Journal 2020; 84(2): 299–359. doi: 10.1253/circj.CJ-19-0773