Eichhorn Andreas, Siepmann Martin, Kirch Wilhelm
Rehabilitationsklinik Nordfriesland, Sankt Peter-Ording, Germany.
Med Klin (Munich). 2010 Jan;105(1):20-5. doi: 10.1007/s00063-010-1003-0. Epub 2010 Feb 3.
Behçet's syndrome rarely occurs in North America and Central Europe (incidence: 1 : 500,000), whereas it is more frequently seen in Japan and Mediterranean countries (incidence: 1 : 10,000). The diagnosis is based on the detection of symptoms and clinical signs. Orogenital aphthosis, anterior and posterior uveitides that frequently cause loss of vision are considered to be primary symptoms. Dermatologic manifestations, i.e., erythema nodosum, vascular lesions (angio-Behçet's syndrome), gastrointestinal ulcers and neurologic involvement, can be observed. HLA B5 is found in some of the patients with Behçet's syndrome. Administration of chlorambucil, a cytotoxic compound, is an effective form of treatment of symptoms and complications of Behçet's syndrome.
The present article describes the course of a female and a male patient who were 39 and 23 years old when Behçet's syndrome was diagnosed for the first time. Treatment with chlorambucil was started in the early 1990s and continued for a period of 9 1/2 and 3(3/4) years, respectively, with the symptoms remitting during and after this therapy. Approximately 10 years after the start of treatment with chlorambucil, the patients' symptoms changed. The female patient who was first diagnosed having Behçet's syndrome at the age of 39 years developed rheumatoid arthritis with joint destruction. Her symptoms could be controlled in the long term by oral administration of prednisone, at doses below the Cushing threshold combined with methotrexate. The male patient who was first diagnosed having Behçet's syndrome at the age of 23 years developed systemic vasculitis remitting during low-dose treatment with prednisone.
Immunosuppressive therapy with chlorambucil administered over several years often induces remission of Behçet's syndrome. However, both case reports indicate that symptoms can change from Behçet's syndrome to systemic vasculitis or rheumatoid arthritis.
白塞病在北美和中欧很少见（发病率：1：500,000），而在日本和地中海国家更为常见（发病率：1：10,000）。诊断基于症状和临床体征的检测。口生殖器口疮、常导致视力丧失的前葡萄膜炎和后葡萄膜炎被认为是主要症状。可观察到皮肤表现，即结节性红斑、血管病变（血管型白塞病）、胃肠道溃疡和神经受累。部分白塞病患者中发现有HLA B5。给予苯丁酸氮芥这种细胞毒性化合物是治疗白塞病症状和并发症的一种有效方法。
本文描述了一名女性和一名男性患者的病程，他们首次被诊断为白塞病时分别为39岁和23岁。20世纪90年代初开始用苯丁酸氮芥治疗，分别持续了9.5年和3又3/4年，治疗期间及之后症状缓解。在开始用苯丁酸氮芥治疗大约10年后，患者症状发生了变化。39岁首次被诊断为白塞病的女性患者出现了伴有关节破坏的类风湿关节炎。通过口服低于库欣阈值剂量的泼尼松联合甲氨蝶呤，她的症状可得到长期控制。23岁首次被诊断为白塞病的男性患者出现了系统性血管炎，在低剂量泼尼松治疗期间缓解。
多年使用苯丁酸氮芥进行免疫抑制治疗常可诱导白塞病缓解。然而，这两个病例报告均表明症状可能从白塞病转变为系统性血管炎或类风湿关节炎。
