Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.
Mod Rheumatol. 2020 Mar;30(2):219-224. doi: 10.1080/14397595.2019.1696504. Epub 2019 Dec 2.
Behçet's disease is a chronic relapsing inflammatory disorder characterized by oral and genital ulcers, skin lesions, and uveitis. Although the involvement of the gastrointestinal, large vascular, and central nervous systems is less common, it can be fatal at times. To suppress inflammatory exacerbations and recurrences which can cause irreversible organ damage, treatment should be individualized according to the disease phenotype and severity. Apremilast, an oral phosphodiesterase 4 inhibitor, is used to treat psoriasis. The enzyme specific inhibitor increases intracellular cAMP levels, which modulate multiple signaling pathways in both innate and adaptive immunocompetent cells, leading to attenuation of inflammatory responses through the suppression of the functions of various types of immunocompetent cells including Th1 cells, Th17 cells and M1 macrophages. Current evidence shows that apremilast is effective for oral ulcer, the most common symptom of Behçet's disease. An oral ulcer is considered to represent the basic immunopathology of Behçet's disease, because the disease susceptibility genes are shared between Behçet's disease and recurrent aphthous stomatitis. The favorable effects have been documented in other inflammatory diseases with symptoms similar to those of Behçet's disease. The further studies would shed the light on the potential of apremilast for the treatment of Behçet's disease.
白塞病是一种慢性复发性炎症性疾病,其特征为口腔溃疡和外阴溃疡、皮肤损伤和葡萄膜炎。尽管胃肠道、大血管和中枢神经系统受累不常见,但有时也可能致命。为了抑制炎症加重和复发,从而避免不可逆转的器官损伤,应根据疾病表型和严重程度进行个体化治疗。阿普米司特是一种口服磷酸二酯酶 4 抑制剂,用于治疗银屑病。这种酶的特异性抑制剂会增加细胞内 cAMP 水平,从而调节固有和适应性免疫细胞中的多种信号通路,通过抑制各种免疫细胞(包括 Th1 细胞、Th17 细胞和 M1 巨噬细胞)的功能来减轻炎症反应。目前的证据表明,阿普米司特对口腔溃疡(白塞病最常见的症状)有效。口腔溃疡被认为代表了白塞病的基本免疫病理学,因为白塞病和复发性阿弗他溃疡的疾病易感基因是共享的。在具有类似于白塞病的症状的其他炎症性疾病中,也有其疗效的相关记录。进一步的研究将揭示阿普米司特治疗白塞病的潜力。