The relationship between malignancy and Behçet's disease features

Year 2022, Volume: 5 Issue: 2, 633 - 639, 15.03.2022

İsmail Doğan , Berkan Armağan , Kevser Gök , Esra Kayacan Erdoğan , Şükran Erten

https://doi.org/10.32322/jhsm.1063623

Abstract

Introduction: Behçet’s disease (BD) is an autoimmune, multisystemic vasculitis characterized by chronic inflammation. Autoimmune responses in BD could drive chronic inflammation which is a risk for malignant transformation. Some genetic, environmental, clinical features and immunosuppressive treatments in BD may increase the risk of malignancy. Common genetic factors and similar environmental factors play a role in the pathogenesis of some autoimmune diseases and malignancies. We hypothesized that the frequency of comorbidity and clinical features of BD may differ in BD patients with a family history of malignancy. So, we aimed to compare the demographic and clinical characteristics features of the BD patients with and without a family history of malignancy.
Material and Method: The BD patients who admitted the rheumatology outpatient clinic consecutively were included in the study. The demographic and clinical characteristics, comorbidities including malignancy in BD patients and malignancies in their family were questioned. The acute phase reactant elevation of at least two follow-ups was accepted as chronic inflammation.
Results: A total of 98 patients (57% male) were included. Mean age was 43.5±12.3 years. The frequency of comorbidity was 60% and malignant/premalignant lesions were seen in 5% of the patients. All lesions were solid organ related and all of them were in women. History of BD and malignancy in patients' families was found 28% and 38%, respectively. The patients with and without malignancy in their family were compared. Female gender and the frequency of erythema nodosum were higher in the patients with malignancy in their family. The other demographic and clinical characteristics, chronic persistent inflammation and medical treatments were statistically. not different
Conclusion: Frequency of malignancy in BD patients’ family was evaluated and to the best of our knowledge, there was no literature data on this subject interestingly. The family history of malignancy in BD patients could be associated with clinical characteristics. Further prospective studies were needed to show the clinical effect of malignancy history in families.

Keywords

Behçet Disease, comorbidity, malignancy, family history

Project Number

yok

References

• Cakir N, Dervis E, Benian O, et al. Prevalence of Behçet's disease in rural western Turkey: a preliminary report. Clin Exp Rheumatol 2004; 22: 53-5.
• Fietta P. Behçet's disease: familial clustering and immunogenetics. Clin Exp Rheumatol 2005; 23: 96-105.
• Yazici Y, Hatemi G, Bodaghi B, et al. Behçet syndrome. Nat Rev Dis Primers 2021; 7: 67.
• Wang LH, Wang WM, Hsu SM, Lin SH, Shieh CC. Risk of overall and site-specific cancers in Behcet disease: a nation wide population-based study in Taiwan. J Rheumatol 2015; 42: 879-84
• Na SJ, Kang MJ, Yu DS, et al. Cancer risk in patients with Behçet disease: A nationwide population-based dynamic cohort study from Korea. J Am Acad Dermatol 2018; 78: 464-70
• Lin Y, Li G, Zheng W, Tian X, Zhang F. Behcet's disease associated with malignancy: a report of 41 Chinese cases. Int J Rheum Dis 2014; 17: 459-65
• Cengiz M, Altundag MK, Zorlu AF, Güllü IH, Ozyar E, Atahan IL. Malignancy in Behçet's disease: a report of 13 cases and a review of the literature. Clin Rheumatol 2001; 20: 239-44.
• Tada Y, Koarada S, Haruta Y, Mitamura M, Ohta A, Nagasawa K. The association of Behçet's disease with myelodysplastic syndrome in Japan: a review of the literature. Clin Exp Rheumatol 2007; 25: 507-8
• Kaklamani VG, Tzonou A, Kaklamanis PG. Behçet's disease associated with malignancies. Report of two cases and review of the literature. Clin Exp Rheumatol 2005; 23: 35-41.
• Gurcan M, Esatoglu SN, Hamuryudan V, et al. Long term follow-up of Behçet's syndrome patients treated with cyclophosphamide. Rheumatology (Oxford) 2020; 59: 2264-71.
• Martin DN, Mikhail IS, Landgren O. Autoimmunity and hematologic malignancies: associations and mechanisms. Leuk Lymphoma 2009; 50: 541-50
• Guven DC, Bolek EC, Altintop SE, et al. Cancer incidence in Behçet's disease. Ir J Med Sci 2020 ; 189: 1209-14.
• Huang MX, Wang CY, Guo JY, et al. Pharmacotherapy for Behçet's Disease and the Risk of Malignancy. Front Pharmacol 2021; 12: 661150.
• . Uçar F, Sönmez M, Ermantaş N, et al. The associations of HLA-A, -B, DRB1 alleles and haplotypes in Turkish lymphoma patients. Gene 2016; 586 : 263-7.
• Xiao X, Liu L, Li WJ, Liu J, Chen DJ. HLA-A, HLA-B, HLA-DRB1 polymorphisms and risk of cervical squamous epithelial cell carcinoma: a population study in China. AsianPac J CancerPrev 2013; 14: 4427-33.
• Shuxian J, Xiaoyun C, Zhihui F, et al. Association of HLA-B*51: 01 with papillary thyroid carcinoma in the Chinese Han population of the Shandong coastal areas. Thyroid 2014; 24: 867-71.
• Sieverding M, Arbogast AL, Zintel S, von Wagner C. Gender differences in self-reported family history of cancer: A review and secondary data analysis. Cancer Med 2020; 9: 7772-80.
• Krakow M, Rising CJ, Trivedi N, Yoon DC, Vanderpool RC. Prevalence and correlates of family cancer history knowledge and communication among US adults. Prev Chronic Dis 2020; 17: 146.
• Smith RA, Andrews KS, Brooks D, et al. Cancer screening in the United States, 2018: A review of current American Cancer Society guidelines and current issues in cancer screening. CA Cancer J Clin 2018; 68: 297-316.
• Criteria for diagnosis of Behçet’s disease. International Study Group for Behçet’s Disease. Lancet 1990; 335: 1078-80.
• Baecklund E, Smedby KE, Sutton LA, Askling J, Rosenquist R. Lymphoma development in patients with autoimmune and inflammatory disorders—whatare the driving forces? Semin Cancer Biol 2014; 24: 61-70.
• Naramala S, Konala VM, Adapa S, et al. Trends in hospitalization and inpatient outcomes of behçet's disease: a nationwide inpatient sample study. Cureus 2020; 12: 7470.
• Wang X, Peng Y, Gao J, Han S, Li Y. Risk of malignancy in Behcet disease: a meta-analysis with systematic review. Medicine (Baltimore) 2019; 98: 17735.
• Na SY. Morbidity of solid cancer in Behcet’s disease: analysis of 11 cases in a series of 506 patients. Yonsei Med J 2013; 54: 895–901.
• Kural-Seyahi E, Fresko I, 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; 82: 60-76.
• Hidaka A, Sawada N, Svensson T, et al; JPHC Study Group. Family history of cancer and subsequent risk of cancer: A large-scale population-based prospective study in Japan. Int J Cancer 2020; 147: 331-7.