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A retrospective study comparing the phenotype and outcomes of patients with polyarteritis nodosa between UK and Turkish cohorts

  • Omer Karadag
  • Abdulsamet Erden
  • Yelda Bilginer
  • Seerapani Gopaluni
  • Alper Sari
  • Berkan Armagan
  • Ihsan Ertenli
  • Seza Ozen
  • David Jayne
Cohort Studies

Abstract

There is a need for better definition of polyarteritis nodosa (PAN) subphenotypes and the influence of ethnicity and geography. This study is aimed to study the demographic and clinical features of PAN cohorts from the UK and Turkey (TR) and to compare and contrast disease characteristics. A retrospective survey of databases from two vasculitis centres between 1990 and 2016 for PAN patients fulfilling the EMEA Vasculitis Classification algorithm. All paediatric-onset adult patients met the Ankara 2008 (EULAR/PReS endorsed) criteria for childhood PAN. Those with typical angiographic and/or histopathologic findings consistent with PAN were included. 93 (M/F: 51/42) patients (UK: 47, TR: 46) were included. Three were HBV-related, 20 (21.5%) had paediatric onset and 16 (16.5%), cutaneous PAN. TR patients had younger age of disease onset 44 (28.5–59.0) vs. 24.5 (11.8–40.5), p = 0.002. Twelve (26%) of TR patients had monogenic disease (Familial Mediterranean Fever association (n = 7), deficiency of adenosine deaminase 2, DADA2, (n = 5). No difference was found in phenotype between paediatric and adult onset patients except for frequency of cutaneous lesions (p = 0.002). During a median 67.5 (32–126) months follow-up, 13 patients died (12.7% in UK vs. 15.2% in Turkish cohorts). No difference was found between two cohorts in relation to relapse rate, death and vasculitis damage index. This study defined a diagnosis of PAN according to the EMEA algorithm. The TR group had a younger age of disease onset and more cases of monogenic disease; however, disease extent, relapse rate, damage index and death rates were similar between groups.

Keywords

Polyarteritis nodosa (PAN) Vasculitis Familial Mediterranean Fever (FMF) Deficiency of adenosine deaminase 2 (DADA2) Paediatric PAN EMEA vasculitis classification 

Notes

Author contributions

OK, AE, YB, SG, AS, BA, İE, SO, DJ Substantial contributions to the conception or design of the work; or the acquisition, analysis, or interpretation of data for the work. OK, AE, YB, SG, AS, BA, İE, SO, DJ Drafting the work or revising it critically for important intellectual content. OK, AE, YB, SG, AS, BA, İE, SO, DJ Final approval of the version to be published. OK, AE, YB, SG, AS, BA, İE, SO, DJ Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

References

  1. 1.
    Kussmaul A, Maier R (1866) Ueber eine bisher nicht beschriebene eigenthu ̈mliche Arterienerkrankung (Periarteritis nodosa), die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskel- l ̈ahmung einhergeht. Dtsch Arch Klin Med 1:484–518Google Scholar
  2. 2.
    Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V et al (2010) 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 62(2):616–626CrossRefPubMedGoogle Scholar
  3. 3.
    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 65:1–11CrossRefGoogle Scholar
  4. 4.
    Tunca M, Akar S, Onen F, Ozdogan H, Kasapcopur O, Yalcinkaya F et al (2005) Familial Mediterranean Fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore) 84:1–11CrossRefGoogle Scholar
  5. 5.
    Kermani TA, Ham EK, Camilleri MJ, Warrington KJ (2012) Polyarteritis nodosa-like vasculitis in association with minocycline use: a single-center case series. Semin Arthritis Rheum 42:213–221CrossRefPubMedGoogle Scholar
  6. 6.
    Navon Elkan P, Pierce SB, Segel R, Walsh T, Barash J, Pedeh S, Zlotogorski A et al (2014) Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy. N Engl J Med 370:921–931CrossRefPubMedGoogle Scholar
  7. 7.
    de Menthon M, Mahr A (2011) Treating polyarteritis nodosa: current state of the art. Clin Exp Rheumatol 29(1 Suppl 64):S110–S116PubMedGoogle Scholar
  8. 8.
    Erden A, Batu ED, Bilgin E, Armagan B, Karadag O, Ozen S et al (2016) Comparing the characteristics of adult and pediatric patients with polyarteritis nodosa. Ann Rheum Dis 75:799Google Scholar
  9. 9.
    Watts R, Lane S, Scott DG et al (2001) Epidemiology of vasculitis in Europe. Ann Rheum Dis 60(12):1156–1157CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Craven A, Robson J, Ponte C, Grayson PC, Suppiah RS, Watts RA et al (2013) ACR/EULAR-endorsed study to develop diagnostic and classification criteria for vasculitis (DCVAS). Clin Exp Nephrol 17:619–621CrossRefPubMedGoogle Scholar
  11. 11.
    Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldinsnes W et al (2007) Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 66:222–227CrossRefPubMedGoogle Scholar
  12. 12.
    De Groot K, Gross WL, Herlyn K, Reinhold-Keller E (2001) Development and validation of a disease extent index for Wegener’s granulomatosis. Clin Nephrol 55:31–38PubMedGoogle Scholar
  13. 13.
    Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O et al (1996) Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore) 75:17–28CrossRefGoogle Scholar
  14. 14.
    Exley AR, Bacon PA, Luqmani RA, Kitas GD, Gordon C, Savage CO et al (1997) Development and initial validation of the vasculitis damage index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum 40:371–380CrossRefPubMedGoogle Scholar
  15. 15.
    Alibaz-Oner F, Koster MJ, Crowson CS, Makol A, Yterberg SR, Salvarani C et al (2017) The clinical spectrum of medium-sized vessel vasculitis. Arthritis Care Res (Hoboken) 69:884–891CrossRefGoogle Scholar
  16. 16.
    Ozen S (2017) The changing face of polyarteritis nodosa and necrotizing vasculitis. Nat Rev Rheumatol 13:381–386CrossRefPubMedGoogle Scholar
  17. 17.
    Mahr A, Guillevin L, Poissonnet M, Aymé S (2004) 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 51:92–99CrossRefPubMedGoogle Scholar
  18. 18.
    Guillevin L, Mahr A, Callard P, Godmer P, Pagnoux C, Leray E et al, French Vasculitis Study Group (2005) Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine (Baltimore) 84:313–322CrossRefGoogle Scholar
  19. 19.
    Gayraud M, Guillevin L, le Toumelin P, Cohen P, Lhote F, Casassus P et al, French Vasculitis Study Group (2001) Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum 44:666–675CrossRefPubMedGoogle Scholar
  20. 20.
    Ribi C, Cohen P, Pagnoux C, Mahr A, Arene JP, Puechal X et al (2010) Treatment of polyarteritis nodosa and microscopic polyangiitis without poor-prognosis factors: a prospective randomized study of one hundred twenty-four patients. Arthritis Rheum 62:1186–1197CrossRefPubMedGoogle Scholar
  21. 21.
    Samson M, Puéchal X, Mouthon L, Devilliers H, Cohen P, Bienvenu B et al, French Vasculitis Study Group (FVSG) (2017) Microscopic polyangiitis and non-HBV polyarteritis nodosa with poor-prognosis factors: 10-year results of the prospective CHUSPAN trial. Clin Exp Rheumatol 35(Suppl 103):176–184PubMedGoogle Scholar
  22. 22.
    Bohn J, Schippers E, Wagner S, Küsters W, Müller J, Meesmann M, Scheppach W (2010) Solitary liver tumor as a manifestation of polyarteritis nodosa. Dtsch Med Wochenschr 135:236–239CrossRefPubMedGoogle Scholar
  23. 23.
    Hutterer M, Steurer M, Höffberger R, Heinfelner JA, Pircher A, Nowosielski M et al (2014) Polyarteritis nodosa complicating multiple myeloma—a case report and review of the literature. Clin Neuropathol 33:143–151CrossRefPubMedGoogle Scholar
  24. 24.
    Mahr A, Heijl C, Le Guenno G, Faurschou M (2013) ANCA-associated vasculitis and malignancy: current evidence for cause and consequence relationships. Best Pract Res Clin Rheumatol 27:45–56CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Vasculitis and Lupus Clinic, Addenbrooke’s HospitalUniversity of CambridgeCambridgeUK
  2. 2.Hacettepe University Vasculitis CentreAnkaraTurkey
  3. 3.Hacettepe Universitesi Tip Fakultesi Romatoloji Bilim DaliAnkaraTurkey

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