The aim of this study was to determine the clinical characteristics of two patients with malignancy related to Behçet's disease. We reviewed literary-reported cases of Behçet's disease related to myelodysplastic syndrome, neoplasm and leukemia. Our own observation included two cases of comorbid conditions of Behçet's disease. The first patient with Behçet's disease had been examined concurrent development of intestinal tumor. The second one was diagnosed to have chromic myeloleukemia. The malignant transformation of Behçet's disease patients, both solid and hematological, is rather rare. Further accumulation of relevant date and its analysis will allow to find out the relationship between malignant disease and chemotherapy in Behçet's disease.
Background It is postulated by some authors that neurological disturbances, thrombosis, ocular lesions and other severe clinical signs of Behcet’s disease (BD) are most common in males (m) than in females (f). Objectives To compare the frequency of main clinical features of BD in males and females from Dagestan ethnic cohort. Methods 85 patients with BD (ISBD criteria) were enrolled (m=63, mean age 31±9,8 years, f=22, mean age 29±8,6 years. M/F ratio was 2,8:1. Disease duration in m was 11,27±7,4 years, in f - 7,8±7,1 years. Results The main features of BD in m and f are summarized and compared in Table 1. Oral aphthosis occurred with the same frequency in m and f and was 98%. Uveitis, retinal vasculitis and skin lesions were more often in men than in women with BD. Moreover, severe symptoms of BD, such as vasculo-Behcet, or venous thrombosis developed more frequently in males. There was no difference in the frequency of central nervous system and gastro-intestinal damage between m and f with BD. Conclusions It is obvious that disease course is more serious in males than in females with BD from Dagestan ethnic cohort. Possibly there is an influence of HLA-B51 positivity. This antigen appeared to be positive in 75% of males and 47% of females. Disclosure of Interest None Declared
Background Behcet's disease (BD) is chronic disorders characterized by multisystem manifestation. Several studies showed regional differences in BD presentation. The data about BD on Caucasus through passed Silk Road is not enough. Objectives The aim of our study is to compare BD in two ethnic cohorts from North Caucasus. Methods A total of 111 consecutive BD patients (82 male and 29 female) seen in the Research Institute of Rheumatology RAMS (Moscow). All of them fulfilled the classification criteria ISGBD [1]. Results The patients were divided into two groups: I – 85 patients from Dagestan, II – 26 patients from Chechnya. Table 1.Demographic data of BD patients Group I Group II p M:F 2.8:1 2.7:1 NS Age at onset, years 21.7±9.6 22.5±8.8 NS Age at diagnosis, years 30.7±9.4 32.3±8.5 NS The frequency of mostclinical manifestations of BD (%) was similar in comparable groups. Percentage of oral aphthae, genital ulcer, skin and eye disease – 96, 79, 75, 53 in I group and 100, 85, 81, 50 – in II group. Number of patients with HLA B51 positivity and familyaggregation observed more offen in Chechenian cogort (81% and 27% vs 69% and 20%) Table 2.BD and gender in two groups (%) Manifestation Patients from Dagestan % Patients from Chechnya % Male Female p≤0,05 Male Female p≤0,05 n=63 n=22 n=19 n=7 Genital ulcers 86 59 0,01 84 86 NS Skin manifestation: 83 55 0,01 95 43 0,004 – Papulopustul (acnae) 52 27 0,04 61 14 0,02 – Erythema nodosum 50 41 NS 47 43 NS Eye disease: 62 27 0,00 58 29 NS Uveitis – posterior 44 23 0,06 42 14 NS Retinal vasculitis 43 9 0,001 34 14 NS Vascular involvement: 40 9 0,00 32 29 NS CNS 10 9 NS 0 0 NS GI 22 23 NS 11 43 NS HLA B51 (+) 75 47 NS 87 67 NS Family aggregation 22 14 NS 32 14 NS The frequency of ocular vascular disease and among the inhabitants of Dagestan appeared to be significantly higher among men. When comparing manifestations of BD in men and women of the Chechen population, significant differences were obtained only for cutaneous manifestations (p<0,04). Conclusions Our results demonstrate no difference between most manifestations of BD in two ethnic populations. In both groups of patients there was male predominance. References International Study group for Behcet's disease: Criteria for Behcet's Lancet, 1990, 335, 1078-1080 Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.2448
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