Pathergy Test in Behçet's Disease

Abstract

Background: The primary aim of the study is to investigate the frequency of pathergy positivity in active BD patients at the time of the first diagnosis. Its secondary aim is to compare the status of pathergy with sociodemographic, clinical and laboratory characteristics.
Materials and Methods: In this study, 110 BHs were analyzed retrospectively. Sociodemographic characteristics (age, gender), clinical findings, PT status, sedimentation (mm/hr) and C-reactive protein (CRP) (mg/L) values of the patients at the time of initial diagnosis were recorded. Age of onset of disease symptoms <40 were accepted as classical onset, patients ≥40 as late-onset BD. Krause Behçet's Disease Clinical Activity Score was used for disease activity.
Results: S PT was found to be positive in 36 (32.7%) of 110 patients with Behçet's disease. In PT positive patients; there was genital ulcer in 31 (86.1%), arthritis in 9(25.0%), uveitis in 7 (19.4%), deep vein thrombosis in 10 (27.8%), papulopustular lesion in 10 (27.8%), erythema nodosum in 6 (16.7%), neuropenchymal involvement in 1 (2.8%) and neurovascular involvement in 1 (2.8%). In PT negative patients, these frequencies were 66 (89.2%), 19 (25.7%), 22 (29.7%), 19 (25.7%), 12 (16.2%), 7 (9.5%), 2 (2.7%) and 1 (1.4%), respectively. The positivity of PT and the frequency of clinical findings were not statistically different (p>0.05). Median value of sedimentation (mm/h) was 16 (2-98), while the median value of CRP (mg/L) was 3.78 (0-126) in PT positive Behçet patients. In PT negative patients, these values were 17.5 (1-108) and 4.5 (0-121), respectively. When the positivity of PT and the median values of sedimentation (mm/hr) and CRP (mg/L) were compared, no significant difference was found (p>0.05). Median value of disease activity total score was 3 (1-6) in PT positive patients, while this median value was 3 (1-7) in PT negative patients. When PT positive and negative patients were compared in terms of disease activity total scores, there was no significant difference (p=0.494). While there were 34 (33.3%) patients with positive PT among classical age-onset patients, there were 2 (25.0%) among those with late onset. When classical and late-onset patients were compared in terms of PT positivity, no significant difference was found (p=1.000).
Conclusions: The benefit of PT positivity in predicting the clinical severity of the disease and regulating treatment for BD is still controversial and requires further research. Perhaps new, standardized test methods that will increase the sensitivity of PT will resolve this uncertainty and make the test more valuable for clinicians.

Keywords

• Behçet's disease
• Disease Activity
• Pathergy Test

Behçet Hastalarında Paterji Testi

Abstract

Amaç: Çalışmanın birincil amacı aktif Behçet Hastalarında (BH), ilk tanı anındaki paterji pozitiflik sıklığını araştırmaktır. İkincil amacı ise paterji durumu ile sosyodemografik, klinik ve laboratuvar özellikleri karşılaştırmaktır. Materyal ve metod: Çalışmada 110 BH retrospektif olarak incelendi. Hastaların ilk tanı anındaki sosyodemografik özellikleri (yaş, cinsiyet), klinik bulguları, paterji test (PT) sonuçları, sedimentasyon (mm/sa) ve C-reaktif protein (CRP) (mg/L) değerleri kayıt edildi. Hastalık semptomlarının başlangıç yaşı <40 olanlar klasik başlangıçlı, ≥40 olanlar geç başlangıçlı BH olarak kabul edildi. Hastalık aktivitesi için Krause Behçet Hastalığı Klinik Aktivite Skoru kullanıldı. Bulgular: Yüz on Behçet hastasının 36’sında (%32.7) PT’nin pozitif olduğu görüldü. PT pozitif hastaların 31’inde (%86.1) genital ülser, 9’unda (%25.0) artrit, 7’sinde (%19.4) üveit, 10’unda (%27.8) derin ven trombozu, 10’unda (%27.8) papulo-püstüler lezyon, 6’sında (%16.7) eritema nodozum, 1’inde (%2.8) nöroparankimal 1’inde de (%2.8) nörovasküler tutulum mevcuttu. PT negatif olan hastalarda bu sıklıklar sırası ile 66(%89.2), 19(%25.7), 22(%29.7), 19(%25.7), 12(%16.2), 7(%9.5), 2(%2.7), 1(%1.4) idi. PT’nin pozitiflik durumu ile klinik bulguların görülme sıklıkları istatistiksel açıdan farklı değildi (p>0.05). PT pozitif hastalarda sedimentasyon (mm/sa) ortanca değeri 16(2-98) iken CRP (mg/L) ortanca değeri 3.78(0-126) idi. PT negatif olanlarda bu değerler sırası ile 17.5(1-108) ve 4.5(0-121) idi. PT’nin pozitiflik durumu ile sedimentasyon (mm/sa) ve CRP (mg/L) ortanca değerleri karşılaştırıldığında anlamlı farklılık görülemedi (p>0.05). PT pozitif hastalarda, hastalık aktivitesi toplam skoru ortanca değeri 3 (1-6) iken PT negatif olanlarda bu ortanca değer 3 (1-7) idi. PT pozitif ve negatif olan hastalar hastalık aktivitesi toplam skorları açısından karşılaştırıldığında anlamlı farklılık yoktu (p=0.494). Klasik yaş başlangıçlı hastalar içerisinde PT’si pozitif olan 34(%33.3) hasta var iken geç yaş başlangıçlı olanlar içerisinde PT’si pozitif olan 2(%25.0) hasta vardı. Klasik ve geç başlangıçlı hastalar, PT pozitiflik sıklıkları açısından karşılaştırıldığında anlamlı farklılık bulunamadı (p=1.000). Sonuç: PT pozitifliğinin hastalığın klinik şiddetini tahmin etmede ve BH için tedaviyi düzenlemede yararı halen tartışmalıdır ve daha fazla araştırma gerekmektedir. Belki de PT’nin duyarlılığını artıracak yeni, standardize test yöntemleri bu belirsizliği çözecek ve klinisyenler için testi daha değerli hale getirecektir.

Keywords

• Behçet hastalığı
• Hastalık Aktivitesi
• Paterji Testi

Supporting Institution

Araştırma kapsamında herhangi bir kurum ya da kuruluştan finansal destek sağlanmamıştır.

References

1. Onder M, Gürer MA. The multiple faces of Behçet's disease and itsa etiological factors. J Eur Acad Dermatol Venereol. 2001 Mar;15(2):126-36. doi: 10.1046/j.1468-3083.2001.00251.x. PMID: 11495519.
2. Saylan T, Mat C, Fresko I, Melikoğlu M. Behçet's disease in the Middle East. Clin Dermatol. 1999 Mar-Apr;17(2):209-23; discussion 105-6. doi: 10.1016/s0738-081x(99)00013-9. PMID: 10330603.
3. Yurdakul S, Hamuryudan V, Yazici H. Behçet syndrome. Curr Opin Rheumatol. 2004 Jan;16(1):38-42. doi: 10.1097/00002281-200401000-00008. PMID: 14673387.
4. Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease. Lancet. 1990 May 5;335(8697):1078-80. PMID: 1970380.
5. Blobner F. Zurrezidivierenden Hypopyoniritis. Z Augenheilkd 1937;91:129-39.
6. Ozluk E, Balta I, Akoguz O, Kalkan G, Astarci M, Akbay G, et al. Histopathologic Study of Pathergy Test in Behçet's Disease. Indian J Dermatol. 2014 Nov;59(6):630. doi: 10.4103/0019-5154.143568. PMID: 25484413; PMCID: PMC4248521.
7. Askari A, Al-Aboosi M, Sawalha A. Evaluation of pathergy test in North Jordan. Clin Rheumatol. 2000;19(3):249-51. doi: 10.1007/s100670050170. PMID: 10870668.
8. Ozarmagan G, Saylan T, Azizlerli G, Ovül C, Aksungur VL. Re-evaluation of the pathergy test in Behçet's disease. Acta Derm Venereol. 1991;71(1):75-6. PMID: 1676225..

9. Sungur G, Hazirolan D, Hekimoglu E, Kasim R, Duman S. Late-onset Behçet's disease: demographic, clinical, and ocular features. Graefes Arch Clin Exp Ophthalmol. 2010 Sep;248(9):1325-30. doi: 10.1007/s00417-010-1399-5. Epub 2010 May 7. PMID: 20449602.
10. Dilşen N, Koniçe M, Aral O, Ocal L, Inanç M, Gül A. 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. 1993 Nov;52(11):823-5. doi: 10.1136/ard.52.11.823. PMID: 8250614; PMCID: PMC1005196.
11. Krause I, Mader R, Sulkes J, Paul M, Uziel Y, Adawi M, et al. Behçet's disease in Israel: the influence of ethnic origin on disease expression and severity. J Rheumatol. 2001 May;28(5):1033-6. PMID: 11361184.
12. Hinkle DE, Wiersma W, Jurs SG. Applied statistics for the behavioral sciences. Houghton Mifflin College Division; 2003.
13. Tunç R, Uluhan A, Melikoğlu M, Ozyazgan Y, Ozdoğan H, Yazici H. A reassessment of the International Study Group criteria for the diagnosis (classification) of Behçet's syndrome. Clin Exp Rheumatol. 2001 Sep-Oct;19(5 Suppl 24):S45-7. PMID: 11760398.
14. Alpsoy E. Behçet's disease: A comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions. J Dermatol. 2016 Jun;43(6):620-32. doi: 10.1111/1346-8138.13381. Epub 2016 Apr 14. PMID: 27075942.
15. Krause I, Molad Y, Mitrani M, Weinberger A. Pathergy reaction in Behçet's disease: lack of correlation with mucocutaneous manifestations and systemic disease expression. Clin Exp Rheumatol. 2000 Jan-Feb;18(1):71-4. PMID: 10728447.
16. Koç Y, Güllü I, Akpek G, Akpolat T, Kansu E, Kiraz S, et al. Vascular involvement in Behçet's disease. J Rheumatol. 1992 Mar;19(3):402-10. PMID: 1578454.
17. Assar S, Sadeghi B, Davatchi F, Ghodsi SZ, Nadji A, Shahram F, et al. The association of pathergy reaction and active clinical presentations of Behçet's disease. Reumatologia. 2017;55(2):79-83. doi: 10.5114/reum.2017.67602. Epub 2017 Apr 28. PMID: 28539679; PMCID: PMC5442298.
18. Gül U, Gönül M. Oral and genital pathergy in Behçet's disease. Dermatology. 2007;215(1):80-1. doi: 10.1159/000102042. PMID: 17587847.
19. Kalay Yildizhan İ, Boyvat A. Diagnostic Sensitivity of Different Applications of Pathergy Test for Behçet's Disease. Arch Rheumatol. 2019 Apr 15;35(1):29-34. doi: 10.5606/ArchRheumatol.2020.7380. PMID: 32637917; PMCID: PMC7322300.
20. Azizlerli G, Köse AA, Sarica R, Gül A, Tutkun IT, Kulaç M, et al. Prevalence of Behçet's disease in Istanbul, Turkey. Int J Dermatol. 2003 Oct;42(10):803-6. doi: 10.1046/j.1365-4362.2003.01893.x. PMID: 14521694.
21. Sequeira FF, Daryani D. The oral and skin pathergy test. Indian J Dermatol Venereol Leprol. 2011 Jul-Aug;77(4):526-30. doi: 10.4103/0378-6323.82399. PMID: 21727709.
22. Davatchi F, Chams-Davatchi C, Ghodsi Z, Shahram F, Nadji A, Shams H, et al. Diagnostic value of pathergy test in Behcet's disease according to the change of incidence over the time. Clin Rheumatol. 2011 Sep;30(9):1151-5. doi: 10.1007/s10067-011-1694-5. Epub 2011 Mar 2. PMID: 21365194.
23. Kutlubay Z, Tüzün Y, Wolf R. The Pathergy Test as a Diagnostic Tool. Skinmed. 2017 Apr 1;15(2):97-104. PMID: 28528602.
24. Rahman S, Daveluy S. Pathergy Test. 2020 Jun 9. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan–. PMID: 32644335.
25. Varol A, Seifert O, Anderson CD. The skin pathergy test: innately useful? Arch Dermatol Res. 2010 Apr;302(3):155-68. doi: 10.1007/s00403-009-1008-9. Epub 2009 Dec 12. PMID: 20012749.
26. Kural-Seyahi E, Fresko I, Seyahi N, Ozyazgan Y, Mat C, Hamuryudan V, et al. The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003 Jan;82(1):60-76. doi: 10.1097/00005792-200301000-00006. PMID: 12544711.
27. Guzelant G, Ozyazgan Y, Mat C, Hamuryudan V, Yazici H, Seyahi E. Clinical Characteristics of Older Age-Onset Behçet Syndrome Patients. Arthritis Rheumatol. 2018; 70 (suppl 10). https://acrabstracts.org/abstract/clinical-characteristics-of-older-age-onset-behcet-syndrome-patients/. Accessed February 20, 2021.