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

Takaharu Ikeda

Article ID: 1452
Vol 6, Issue 2, 2022

VIEWS - 757 (Abstract) 277 (PDF)

Abstract


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

Full Text:

PDF


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




DOI: https://doi.org/10.24294/ti.v6.i2.1452

Refbacks

  • There are currently no refbacks.


Copyright (c) 2022 Takaharu Ikeda

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

This site is licensed under a Creative Commons Attribution 4.0 International License.