Colpak Ayşe İlksen, Kalyoncu Umut, Gürsoy Özdemir Yasemin
Department of Neurology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Department of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Noro Psikiyatr Ars. 2014 Jun;51(2):141-147. doi: 10.4274/npa.y6987. Epub 2014 Jun 1.
Behçet's disease is a chronic inflammatory disease of unknown aetiology that affects multiple organ systems. Since the diagnosis of this disease mainly relies on clinical criteria, a diagnostic laboratory test is required especially for neuro-Behçet's patients without systemic involvement.
In this study, we searched for the presence of autoantibodies against brain tissue, by means of indirect immunofluorescent staining technique in sera obtained from patients with neuro-Behçet's disease, based on reports that humoral immune dysregulation may play a role in susceptibility to Behçet's disease. After pre-absorbtion of sera with guinea pig liver powder to reduce nonspecific staining, serum samples were applied to mouse brain sections and immunoreactivity was detected with fluorescein (FITC)-conjugated goat antibody against human IgG.
Ten sera from neuro-Behçet's patients and 10 age-matched control sera were screened for immunoreactivity. We detected specific immunoreactivity to both parenchymal and vascular brain structures in the patients' sera. Parenchymal vessel immunopositivity was detected in 8 of 10 patients, whereas only two of control sera showed no significant parenchymal vascular immunoreactivity (p=.025). In addition to vascular immunoreactivity, filamentous and reticular immunopositive structures were detected in brain sections of 5 out of 10 patients. No such immunoreactivity was detected in sections incubated with control sera (p=.016).
We detected a specific immunoreactivity against vascular and parenchymal filamentous structures in neuro-Behçet patients' sera. Humoral autoimmunity may play a role in the pathogenesis of neuro-Behçet's disease in addition to cellular immune response. Findings of this preliminary study will be evaluated with a large number of patients and controls, to determine whether it is the cause or the result and, further studies are underway to disclose the nature of epitope to which the immunoreactivity was directed against and to develop a diagnostic laboratory method for investigating central nervous system involvement in Behçet's patients.
白塞病是一种病因不明的慢性炎症性疾病，可累及多个器官系统。由于该疾病的诊断主要依赖临床标准，因此对于无全身受累的神经白塞病患者，需要一种诊断性实验室检测方法。
在本研究中，基于体液免疫失调可能在白塞病易感性中起作用的报道，我们采用间接免疫荧光染色技术，在神经白塞病患者血清中寻找抗脑组织自身抗体。在用豚鼠肝粉预吸收血清以减少非特异性染色后，将血清样本应用于小鼠脑切片，并用异硫氰酸荧光素（FITC）偶联的抗人IgG山羊抗体检测免疫反应性。
对10份神经白塞病患者血清和10份年龄匹配的对照血清进行免疫反应性筛查。我们在患者血清中检测到对脑实质和脑血管结构的特异性免疫反应性。10例患者中有8例检测到脑实质血管免疫阳性，而对照血清中只有2例未显示明显的脑实质血管免疫反应性（p = 0.025）。除血管免疫反应性外，10例患者中有5例的脑切片中检测到丝状和网状免疫阳性结构。在与对照血清孵育的切片中未检测到此类免疫反应性（p = 0.016）。
我们在神经白塞病患者血清中检测到针对血管和脑实质丝状结构的特异性免疫反应性。除细胞免疫反应外，体液自身免疫可能在神经白塞病的发病机制中起作用。这项初步研究的结果将在大量患者和对照中进行评估，以确定其是病因还是结果，并且正在进行进一步研究以揭示免疫反应性所针对的表位的性质，并开发一种诊断性实验室方法来研究白塞病患者的中枢神经系统受累情况。
