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The diagnostic role of pathergy test in patients with Behçet’s disease from the Western Europe

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Abstract

The aim of the study is to evaluate the frequency and features of positive pathergy test (PPT) in Italy, its role in the diagnosis of Behçet’s disease (BD), and any association with other BD-related manifestations. 52 BD patients, 52 patients with axial spondyloarthritis (ax-SpA), and 26 healthy controls (HCs) underwent intradermal injection of normal saline and intradermal needle soaked with fresh self-saliva. The results of pathergy tests were statistically analysed in the light of demographic, clinical, and therapeutic features of subjects enrolled. Pathergy test performed with saline resulted always negative in all groups. Skin prick test using self-saliva resulted in the occurrence of a papule in 3 (5.8%) BD patients and in 1 (1.9%) patient with ax-SpA. A ≥ 15 mm erythematous area surrounding the needle prick site was observed in 22 (42.3%) BD patients, 5 (9.6%) patients with ax-SpA, and 2 (7.7%) HCs (p = 0.00002). The frequency of skin erythema was significantly more frequent in patients with BD than those with ax-SpA (p < 0.0001) and HCs (p = 0.003). No statistically significant differences were observed between ax-SpA patients and HCs (p = 1.000). The occurrence of skin erythema at pathergy test was not associated with any BD-related clinical manifestation. Erythema at self-saliva prick test presented a sensitivity of 42.31% (CI 28.73–56.80%) and a specificity of 91.03% (CI 82.38–96.32%). The development of a ≥ 15 mm erythematous area at self-saliva prick test could be sufficient to unveil the hyper-reactivity of the innate immune system in BD patients from Western Europe, where the development of skin erythema shows good sensitivity and specificity toward the diagnosis of BD.

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References

  1. Vitale A, Rigante D, Lopalco G, Emmi G, Bianco MT, Galeazzi M, Galeazzi M, Iannone F, Cantarini L (2016) New therapeutic solutions for Behçet’s syndrome expert. Opin Investig Drugs 25:827–840. https://doi.org/10.1080/13543784.2016.1181751

    Article  CAS  Google Scholar 

  2. Yazici Y, Hatemi G, Bodaghi B, Cheon JH, Suzuki N, Ambrose N, Yazici H (2021) Behçet syndrome. Nat Rev Dis Primers 7:67. https://doi.org/10.1038/s41572-021-00301-1

    Article  PubMed  Google Scholar 

  3. Criteria for diagnosis of Behçet’s disease (1990) International Study group for Behçet’s disease. Lancet 335:1078–1080

    Google Scholar 

  4. International Team for the Revision of the International Criteria for Behçet’s Disease (ITR-ICBD) (2014) The International Criteria for Behçet’s disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol 28:338–347. https://doi.org/10.1111/jdv.12107

    Article  Google Scholar 

  5. Gül A, Esin S, Dilsen N, Koniçe M, Wigzell H, Biberfeld P (1995) Immunohistology of skin pathergy reaction in Behçet’s disease. Br J Dermatol 132:901–907. https://doi.org/10.1111/j.1365-2133.1995.tb16946.x

    Article  PubMed  Google Scholar 

  6. Sequeira FF, Daryani D (2011) The oral and skin pathergy test. Indian J Dermatol Venereol Leprol 77:526–530. https://doi.org/10.4103/0378-6323.82399

    Article  PubMed  Google Scholar 

  7. Dilşen N, Koniçe M, Aral O, Ocal L, Inanç M, Gül A (1993) Comparative study of the skin pathergy test with blunt and sharp needles in Behçet’s disease: confirmed specificity but decreased sensitivity with sharp needles. Ann Rheum Dis 52:823–825. https://doi.org/10.1136/ard.52.11.823

    Article  PubMed  PubMed Central  Google Scholar 

  8. Davatchi F, Shahram F, Chams C, Chams H, Nadji A (2005) Behçet’s Disease. Acta Med Iran 43:233–242

    Google Scholar 

  9. Togashi A, Saito S, Kaneko F, Nakamura K, Oyama N (2011) Skin prick test with self-saliva in patients with oral aphthoses: a diagnostic pathergy for Behcet’s disease and recurrent aphthosis. Inflamm Allergy Drug Targets 10:164–170. https://doi.org/10.2174/187152811795564109

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Sieper J, Rudwaleit M, Baraliakos X, Brandt J, Braun J, Burgos-Vargas R, Dougados M, Hermann KG, Landewé R, Maksymowych W, van der Heijde D (2009) The Assessment of spondyloarthritis international society (ASAS) handbook: a guide to assess spondyloarthritis. Ann Rheum Dis. https://doi.org/10.1136/ard.2008.104018

    Article  PubMed  Google Scholar 

  11. Shenavandeh S, Sadeghi SMK, Aflaki E (2021) Pathergy test with a 23G needle with and without self-saliva in patients with Behçet’s disease, recurrent aphthous stomatitis and control group compared to the 20G test. Reumatologia 59:302–308. https://doi.org/10.5114/reum.2021.110567

    Article  PubMed  PubMed Central  Google Scholar 

  12. Varol A, Seifert O, Anderson CD (2010) The skin pathergy test: innately useful? Arch Dermatol Res 302:155–168. https://doi.org/10.1007/s00403-009-1008-9

    Article  PubMed  Google Scholar 

  13. Lawton G, Bhakta BB, Chamberlain MA, Tennant A (2004) The Behcet’s disease activity index. Rheumatology (Oxford) 43:73–78. https://doi.org/10.1093/rheumatology/keg453

    Article  CAS  PubMed  Google Scholar 

  14. Davatchi F, Chams-Davatchi C, Ghodsi Z, Shahram F, Nadji A, Shams H, Akhlaghi M, Larimi R, Sadeghi-Abdolahi B (2011) Diagnostic value of pathergy test in Behcet’s disease according to the change of incidence over the time. Clin Rheumatol 30:1151–1155. https://doi.org/10.1007/s10067-011-1694-5

    Article  PubMed  Google Scholar 

  15. Yazici H, Chamberlain MA, Tüzün Y, Yurdakul S, Müftüoglu A (1984) A comparative study of the pathergy reaction among turkish and british patients with Behçet’s disease. Ann Rheum Dis 43:74–75. https://doi.org/10.1136/ard.43.1.74

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Mahr A, Belarbi L, Wechsler B, Jeanneret D, Dhote R, Fain O, Lhote F, Ramanoelina J, Coste J, Guillevin L (2008) Population-based prevalence study of Behçet’s disease: differences by ethnic origin and low variation by age at immigration. Arthritis Rheum 58:3951–3959. https://doi.org/10.1002/art.24149

    Article  PubMed  Google Scholar 

  17. Bergamo S, di Meo N, Stinco G, Bonin S, Trevisini S, Trevisan G (2017) Adamantiades-Behçet disease at the beginning of the silk route: north-east italian experience. Acta Dermatovenerol Croat 25:295–297

    PubMed  Google Scholar 

  18. Leccese P, Yazici Y, Olivieri I (2017) Behcet’s syndrome in nonendemic regions. Curr Opin Rheumatol 29:12–16. https://doi.org/10.1097/BOR.0000000000000349

    Article  PubMed  Google Scholar 

  19. Askari A, Al-Aboosi M, Sawalha A (2000) Evaluation of pathergy test in north jordan. Clin Rheumatol 19:249–251. https://doi.org/10.1007/s100670050170

    Article  CAS  PubMed  Google Scholar 

  20. Moll JM, Haslock I, Macrae IF, Wright V (1974) Associations between ankylosing spondylitis, psoriatic arthritis, Reiter’s disease, the intestinal arthropathies, and Behcet’s syndrome. Medicine (Baltimore) 53:343–364. https://doi.org/10.1097/00005792-197409000-00002

    Article  CAS  PubMed  Google Scholar 

  21. McGonagle D, Aydin SZ, Gül A, Mahr A, Direskeneli H (2015) ’MHC-I-opathy’-unified concept for spondyloarthritis and Behçet disease. Nat Rev Rheumatol 11:731–740. https://doi.org/10.1038/nrrheum.2015.147

    Article  CAS  PubMed  Google Scholar 

  22. Vitale A, Rigante D, Lopalco G, Selmi C, Galeazzi M, Iannone F, Cantarini L (2016) Interleukin-1 Inhibition in Behçet’s disease. Isr Med Assoc J 18:171–176

    PubMed  Google Scholar 

  23. Mauro D, Thomas R, Guggino G, Lories R, Brown MA, Ciccia F (2021) Ankylosing spondylitis: an autoimmune or autoinflammatory disease? Nat Rev Rheumatol 17:387–404. https://doi.org/10.1038/s41584-021-00625-y

    Article  CAS  PubMed  Google Scholar 

  24. Hsieh J, Kadavath S, Efthimiou P (2014) Can traumatic injury trigger psoriatic arthritis? a review of the literature. Clin Rheumatol 33:601–608. https://doi.org/10.1007/s10067-013-2436-7

    Article  PubMed  Google Scholar 

  25. Camargo CM, Brotas AM, Ramos-e-Silva M, Carneiro S (2013) Isomorphic phenomenon of Koebner: facts and controversies. Clin Dermatol 31:741–749. https://doi.org/10.1016/j.clindermatol.2013.05.012

    Article  PubMed  Google Scholar 

  26. Kalay Yildizhan İ, Boyvat A (2019) Diagnostic sensitivity of different applications of pathergy test for Behçet’s disease. Arch. Rheumatol. 35:29–34. https://doi.org/10.5606/ArchRheumatol.2020.7380

    Article  PubMed  PubMed Central  Google Scholar 

  27. Assar S, Sadeghi B, Davatchi F, Ghodsi SZ, Nadji A, Shahram F, Ashofte F, Larimi SR, Sadeghi M (2017) The association of pathergy reaction and active clinical presentations of Behçet’s disease. Reumatologia 55:79–83. https://doi.org/10.5114/reum.2017.67602

    Article  PubMed  PubMed Central  Google Scholar 

  28. Alli N, Gur G, Yalcin B, Hayran M (2009) Patient characteristics in Behçet disease: a retrospective analysis of 213 Turkish patients during 2001–4Am. J Clin Dermatol 10:411–418. https://doi.org/10.2165/11310880-000000000-00000

    Article  Google Scholar 

  29. Krause I, Molad Y, Mitrani M, Weinberger A (2000) Pathergy reaction in Behçet’s disease: lack of correlation with mucocutaneous manifestations and systemic disease expression. Clin Exp Rheumatol 18:71–74

    CAS  PubMed  Google Scholar 

  30. Davies PG, Fordham JN, Kirwan JR, Barnes CG, Dinning WJ (1984) The pathergy test and Behçet’s syndrome in Britain. Ann Rheum Dis 43:70–73. https://doi.org/10.1136/ard.43.1.70

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Gürler A, Boyvat A, Türsen U (1997) Clinical manifestations of Behçet’s disease: an analysis of 2147 patients. Yonsei Med J 38:423–427. https://doi.org/10.3349/ymj.1997.38.6.423

    Article  PubMed  Google Scholar 

  32. Al-Rawi ZS, Sharquie KE, Khalifa SJ, Al-Hadithi FM, Munir JJ (1986) Behçet’s disease in Iraqi patients. Ann Rheum Dis 45:987–990. https://doi.org/10.1136/ard.45.12.987

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. Chang HK, Cheon KS (2002) The clinical significance of a pathergy reaction in patients with Behcet’s disease. J Korean Med Sci 17:371–374. https://doi.org/10.3346/jkms.2002.17.3.371

    Article  PubMed  PubMed Central  Google Scholar 

  34. Akmaz O, Erel A, Gürer MA (2000) Comparison of histopathologic and clinical evaluations of pathergy test in Behçet’s disease. Int J Dermatol 39:121–125. https://doi.org/10.1046/j.1365-4362.2000.00884.x

    Article  CAS  PubMed  Google Scholar 

  35. Kutlubay Z, Tüzün Y, Wolf R (2017) The Pathergy test as a diagnostic tool. Skinmed 1(15):97–104

    Google Scholar 

  36. Vitale A, Della Casa F, Ragab G, Almaghlouth IA, Lopalco G, Pereira RM, Guerriero S, Govoni M, Sfikakis PP, Giacomelli R, Ciccia F, Monti S, Ruscitti P, Piga M, Lomater C, Tufan A, Opris-Belinski D, Emmi G, Hernández-Rodríguez J, Şahin A, Sebastiani GD, Bartoloni E, Akkoç N, Gündüz ÖS, Cattalini M, Conti G, Hatemi G, Maier A, Parronchi P, Del Giudice E, Erten S, Insalaco A, Li Gobbi F, Maggio MC, Shahram F, Caggiano V, Hegazy MT, Asfina KN, Morrone M, Prado LL, Dammacco R, Ruffilli F, Arida A, Navarini L, Pantano I, Cavagna L, Conforti A, Cauli A, Marucco EM, Kucuk H, Ionescu R, Mattioli I, Espinosa G, Araújo O, Karkaş B, Canofari C, Sota J, Laymouna AH, Bedaiwi AA, Colella S, Giardini HAM, Albano V, Lo Monaco A, Fragoulis GE, Kardas RC, Berlengiero V, Hussein MA, Ricci F, La Torre F, Rigante D, Więsik-Szewczyk E, Frassi M, Gentileschi S, Tosi GM, Dagostin MA, Mahmoud AAA, Tarsia M, Alessio G, Cimaz R, Giani T, Gaggiano C, Iannone F, Cipriani P, Mourabi M, Spedicato V, Barneschi S, Aragona E, Balistreri A, Frediani B, Fabiani C, Cantarini L, Alliance AD, (AIDA) Network, (2022) Development and implementation of the AIDA international registry for patients with Behçet’s disease. Intern Emerg Med. https://doi.org/10.1007/s11739-022-03038-1

    Article  PubMed  PubMed Central  Google Scholar 

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Correspondence to Luca Cantarini.

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The study has been approved by the local Ethics Committee of Azienda Ospedaliera Universitaria Senese, Siena, Italy (AIDA Project; Ref. No.14,951). The study protocol conformed to the tenets of the Declaration of Helsinki; informed consent was obtained from all patients and HC enrolled.

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Informed consent was obtained from all patients and healthy controls enrolled; animals have not been included in the present study.

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The original online version of this article was revised: In this article the author name is wrongly mentioned Vitale Antonio instead of Antonio Vitale. It has been corrected.

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Vitale, A., Berlengiero, V., Caggiano, V. et al. The diagnostic role of pathergy test in patients with Behçet’s disease from the Western Europe. Intern Emerg Med 18, 77–83 (2023). https://doi.org/10.1007/s11739-022-03117-3

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