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Spring 2014, Vol. 22 No. 1

Hong Kong J. Dermatol. Venereol. (2014) 22, 5-11


Original Article

Activation parameters in Behçt's disease

白塞氏病的活躍參數

B Tursen, R Alp, U Tursen, TI Kaya

Abstract

Objective: Behçt's disease (BD) is a chronic, multi-system vasculitis of unknown aetiology. Specific criteria to assess the activity of the disease, which has a relapsing course, have not been found, thus evaluation of the disease activity is mainly based on the clinical findings. Therefore, there is a need for laboratory markers that can be correlated with the disease activity. In this report, we aimed to search for parameters that may be used as an activation marker in BD. Methods: Serum levels of acute phase reactants were evaluated in active and inactive Behçt's disease patients. Mutations of factor V Leiden (FVL), prothrombin (PT) and methylenetetrahydrofolate reductase (MTHFR) were analysed and compared according to systemic involvement in patients with BD. Results: Erythrocyte sedimentation rate (ESR) was found to be higher in the active group, whereas it was lower in the inactive group. There was a statistically significant difference between these data (p=0.023). There was no significant difference among other parameters between these two groups which included D-dimer, C-reactive protein, fibrinogen and procalcitonin levels. The FVL mutation was detected in 25% of the BD patients with vascular involvement compared with 0% of the BD patients without vascular involvement. The difference was statistically significant (p<0.022). However, the prevalence of the PT and MTHFR mutations was normal in patients with BD according to systemic involvement. Conclusion: Results of the present study suggested that FVL mutation might be an additional risk factor for the development of vascular involvement in BD and increased ESR level was associated with active BD.

目標:白塞氏病是一種成因不明的慢性多系統血管炎,病情多反覆發作,現行並沒有一套準繩可靠的標準來評估其疾病的活躍度,病情評估只能建基於其臨床病徵。因此,有需要去發掘可對應臨床病情的實驗室指標。在這份報告中,我們的目標是要發掘 出可作為白塞氏病活躍指標的參數。方法:我們分別對病情活躍與不活躍的白塞氏病患者進行了急性期反應物的血清水平評估;並同時分析及比較萊登第五因子、凝血酶原和甲基四氫葉酸還原酶的基因突變率在不同程度的白塞氏病全身受累患者間的異同。結果:紅細胞沉降率在白塞氏病活躍群組較病情不活躍群組為高,其數據在統計學上有顯著的差異(p=0.023)。在以上兩群組間,其他的研究參數包括D-二聚體、C-反應蛋白、纖維蛋白原及降鈣素原的血清水平則未發現有顯著差異。另外,在百分之二十五有血管受累的白塞氏病患者被發現帶有萊登第五因子基因的突變,無血管受累群組中則沒有發現,兩組的差異在統計學上是顯著的(p<0.022)。此外,凝血酶原和甲基四氫葉酸還原酶的基因突變盛行率在對比白塞氏病全身受累不同程度的患者間則沒有分別。總結:本研究結果顯示萊登第五因子基因突變可能是白塞氏病血管受累的新發現風險因素,另外紅細胞沉降率水平的增加亦與白塞氏病的病情活躍度有關聯。

Keywords: Activation, Behçt's disease, parameters

關鍵詞: 活躍,白塞氏病,參數