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草稿:贝赛特氏症

维基百科，自由的百科全书
贝赛特氏症
异名
  • 贝赛特氏病（Behçet disease）
  • 贝赛特氏症（Behçet's syndrome）
  • 丝路病（Silk Road disease）
前房葡萄膜炎为贝赛特氏症的典型特征，图中可以看到前房蓄脓（英语：hypopyon）。
读音
  • /bɛˈtʃɛt/, 土耳其语：[behˈtʃet]
症状口腔溃疡、生殖器溃疡、葡萄膜炎、关节炎[1]、慢性疲劳
并发症视觉障碍、关节炎、血栓、动脉瘤[2]
起病年龄20至40岁[1][2]
病程长期[1]
病因不明[1]
诊断方法症状学诊断[1]
鉴别诊断反应性关节炎、史蒂芬斯-强森症候群、急性发热性嗜中性皮病[2]
药物皮质类固醇等免疫抑制剂[1]
预后有时会缓解[2]
患病率亚裔常见，尤见于丝路地带，欧美罕见[2]。
分类和外部资源
医学专科风湿病学、免疫学
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贝西特氏症（Behçet's disease，BD）是一种会影响身体多个部位的发炎性及病[2]。最常见的症状包括口腔及其他部位黏膜出现疼痛性溃疡、眼部炎症，以及关节炎[1][2]。溃疡可能持续数日，甚至一周以上。较少见的情况下，可能会出现脑部或脊髓的炎症、血栓、动脉瘤或视觉障碍[1][2]。这些症状通常呈现反复发作的情形[1]。

目前病因不明[1]，可能与基因疾患有关[2]。贝西特氏症并非传染病[1]。诊断需根据一年内至少三次口腔溃疡发作，以及下列条件中至少两项：生殖器溃疡、眼部发炎、皮肤病变、皮肤针刺试验（英语：Pathergy test）呈阳性反应[1]。

目前尚无治愈性疗法。治疗方式可能包括免疫抑制剂（如皮质类固醇与抗肿瘤坏死因子药物）及生活方式调整[1]。 利多卡因漱口水可帮助缓解疼痛[2]。秋水仙素可降低发作频率[2]。

此病在中东与亚洲则较常见，在美国与欧洲较为少见。例如在土耳其，每千人中约有两人罹患此病[2]。好发年龄通常为二十至四十岁之间[1][2]。该病最早于1937年由土耳其皮肤科医师胡鲁西·贝西特（英语：Hulusi Behçet）所描述。[3]

症状

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皮肤与黏膜

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几乎所有贝西特氏症患者皆会出现某种形式的口腔疼痛性溃疡[4]。这些溃疡属于阿弗他口炎，为非疤痕性的口腔病灶。 其病灶与炎症性肠病相似，并可能反复发作[4]。生殖器溃疡常出现在肛门、外阴或阴囊周围，75%的患者会留下疤痕。 此外，患者也可能出现结节性红斑、脓疱性血管炎或类似坏疽性脓皮症的皮肤病灶[4]。

眼睛

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Left eye blood vessels
以左眼视神经乳头（英语：optic disc）为中心的眼底照影。

眼部发炎可能在疾病早期即发生，且20%的病例可能永久性视力丧失。眼部病变可能包括葡萄膜炎（后葡萄膜炎或前葡萄膜炎）、视网膜血管炎（英语：retinal vasculitis）。前葡萄膜炎表现为眼痛、结膜充血、前房积脓（英语：hypopyon）与视力下降；后葡萄膜炎则以无痛性视力下降与飞蚊症为主。视网膜血管炎为较罕见的表现，可导致无痛性视力减退，伴随飞蚊症或视野缺损[4]。

视神经进犯较罕见，通常以进行性视神经萎缩（英语：optic atrophy）与视力丧失呈现，也有报告指出急性视神经病变（尤其是前缺血性视神经病变（英语：anterior ischemic optic neuropathy））的案例。[5] 贝西特氏症可造成原发性或继发性视神经病变。若因脑硬膜静脉窦血栓或视网膜病变导致的视神经萎缩，则属于继发性[6][7]。

Signs and symptoms of acute optic neuropathy（英语：optic neuropathy） include painless loss of vision which may affect either one or both eyes, reduced visual acuity, reduced color vision, relative afferent pupillary defect（英语：relative afferent pupillary defect）, central scotoma（英语：scotoma）, swollen optic disc, macular edema, or retrobulbar pain. When these symptoms occur with concurrent mucocutaneous ulcerations, they raise suspicion of acute optic neuropathy in Behçet's Disease. Progressive optic atrophy may result in decreased visual acuity or color vision. Intracranial hypertension with papilledema may be present.[8]

Episcleritis（英语：Episcleritis） may occur, which causes eye redness and mild pain, without a significant impact on vision.[9]




急性视神经病变（英语：optic neuropathy）的症状包括单侧或双侧无痛性视力丧失、视力与色觉下降、相对传入性瞳孔缺陷（英语：relative afferent pupillary defect）、中央暗点（英语：scotoma）、视神经盘肿胀、黄斑水肿，或眼窝后疼痛。当这些症状与黏膜溃疡同时出现时，须高度怀疑贝西特氏症导致的急性视神经病变。进行性视神经萎缩会导致视力与色觉下降，并可能合并颅内压升高与视乳头水肿。Behçet syndrome. Yazıcı, Yusuf., Hatemi, Gulen., Seyahi, Emire., Yazici, Hasan. 2nd. Cham: Springer. 2020: 77. ISBN 978-3-030-24131-5. OCLC 1127393738. 

结膜上巩膜炎（英语：Episcleritis）亦可能出现，造成眼红与轻微疼痛，但对视力影响不大。Schonberg, Stacy; Stokkermans, Thomas J. Episcleritis. StatPearls Publishing. 2023-08-07 [2024-07-02]. PMID 30521217. 

肠道

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Gastrointestinal (GI) manifestations include abdominal pain, nausea, and diarrhea with or without blood, and they often involve the terminal ileum and ileocecal valve.[4] Some patients with BD experience abdominal tenderness, bloating, and general abdominal discomfort. When mild this can resemble irritable bowel syndrome; more severe cases bear similarities to inflammatory bowel diseases such as ulcerative colitis or Crohn's. Behçet's disease causes ulcers in the terminal ileum and ileocecal valve. The ulcers may be aphthous or have a classic punched out appearance with undermining. Linear and fissuring ulcers up to 5 cm may be present. Biopsies show vasculitis (phlebitis or venulitis) with a neutrophilic inflammatory infiltrate. Involvement of the oesophagus, stomach and large intestine is rare.[来源请求]

肠胃道表现包括腹痛、恶心与腹泻（可伴随血便），通常侵犯末端回肠与回盲瓣。 有些患者会感到腹部压痛、胀气与不适。轻度时类似肠躁症，严重时则与溃疡性结肠炎或克隆氏症相似。贝西特氏症导致末端回肠与回盲瓣溃疡，呈阿弗他型或典型穿凹型，最大可达5公分，且伴有线状或裂隙状溃疡。病理检查可见嗜中性细胞性血管炎（静脉炎或小静脉炎）。食道、胃与大肠受累相对少见。[来源请求]

肺脏

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Lung involvement is typically in the form of hemoptysis, pleuritis, cough, or fever, and in severe cases can be life-threatening if the outlet pulmonary artery develops an aneurysm which ruptures causing severe vascular collapse and death from bleeding in the lungs.[4] Pulmonary artery thrombosis may occur.

肺部受累表现包括咳血、胸膜炎、咳嗽与发烧，严重者可能因肺动脉瘤破裂导致致命性出血与血管崩解。 亦可能出现肺动脉血栓形成。

关节

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Arthritis is seen in up to half of people, and is usually a non-erosive poly or oligoarthritis primarily of the large joints of the lower extremities.[4]

多达一半患者出现关节炎，通常为非侵蚀性之多关节炎或少关节炎，以下肢大关节为主。

肾脏

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贝西特氏症少见引起肾脏病变，可能表现为：


Drug side effects, such as NSAIDs (non-steroidal anti-inflammatories), cyclophosphamide, cyclosporine and tacrolimus. Small vessel vascular disease results in renal vasculitis, whereas large vessel involvement causes aneurysms (bulging) and thrombosis (blockages). Serious kidney problems are more common in men typically with a history of large vessel involvement in other parts of the body. Bladder and urethral involvement is rare in Behçet's disease.

小血管病变导致肾血管炎，大血管病变则可能出现动脉瘤或血栓形成。男性患者、且有其他部位大血管病变病史者较易发生严重肾病。膀胱与尿道受累相对少见。

脑部

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主条目：神经性贝西特氏症

Central nervous system (CNS) involvement most often occurs as a chronic meningoencephalitis. Lesions tend to occur in the brainstem, the basal ganglia and deep hemispheric white matter and may resemble those of multiple sclerosis (MS). Brainstem atrophy is seen in chronic cases.[来源请求]

Neurological involvements range from aseptic meningitis（英语：aseptic meningitis） to vascular thrombosis such as dural sinus thrombosis and organic brain syndrome（英语：organic brain syndrome） manifesting with confusion, seizures, and memory loss. Sudden hearing loss (sensorineural) is often associated with it.[4] They often appear late in the progression of the disease but are associated with a poor prognosis.[来源请求]

中枢神经系统病变最常见为慢性脑膜脑炎，病灶常分布于脑干、基底核与深层白质，影像类似多发性硬化症，慢性期可能出现脑干萎缩。[来源请求]

神经系统病变包括无菌性脑膜炎与血管性病变，如脑静脉窦血栓形成与器质性脑症候群（英语：organic brain syndrome），表现为意识混乱、癫痫与记忆丧失。突发性感觉神经性听力丧失亦常见。 通常出现在疾病后期，预后不良。[来源请求]

心脏

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Chronic aortic regurgitation due to aortic root disease may also be seen.[10] Although infrequent, myocardial infarction (heart attack) with angiographically identified acute coronary artery thrombosis has been reported, including one case with a pathologically demonstrable lesion due to arteritis found at autopsy.[11]

由主动脉根部病变导致的慢性主动脉闭锁不全可能出现。Adam C. Ring. Steven S. Agabegi; Elizabeth Agabegi , 编. Step-up to medicine 3rd. Philadelphia: Wolters Kluwer/Lippincott Williams & Wilkins. 2012-08-23: 266. ISBN 978-1-60913-360-3.  虽然罕见，但也曾报告因急性冠状动脉血栓形成导致心肌梗死的案例，包括一例在病理解剖中证实动脉炎病灶者。Schiff, Steven; Moffatt, Roy; Mandel, William J.; Rubin, Stanley A. Acute myocardial infarction and recurrent ventricular arrhythmias in Behcet's syndrome需要付费订阅. American Heart Journal. 1982, 103 (3): 438–440. PMID 7064781. doi:10.1016/0002-8703(82)90289-7. 

血管

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Blood vessel problems are observed in 7–29% of people with arterial lesions representing 15% of vascular lesions. Arterial lesions pose a greater risk. Most common arterial lesions are occlusions or stenosis and aneurysms or pseudoaneurysms.[12] [来源请求]

血管病变发生率约为7–29%，其中动脉病变占血管病变的15%。动脉病变风险较高，常见表现包括阻塞、狭窄、动脉瘤与伪性动脉瘤。ElGuindy MS, ElGuindy AM. Aneurysmal coronary artery disease: An overview. Global Cardiology Science and Practice (Glob Cardiol Sci Pract.). October 2017, 2017 (3): e201726. PMC 5856968 可免费查阅. PMID 29564347. doi:10.21542/gcsp.2017.26 可免费查阅.  [来源请求]

Cause

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The cause is not well-defined, but it is primarily characterized by auto-inflammation of the blood vessels. Although sometimes erroneously referred to as a diagnosis of exclusion, the diagnosis can sometimes be reached by pathologic examination of the affected areas.[13]

The primary mechanism of the damage is autoimmune, which by definition is an overactive immune system that targets the patient's own body. The involvement of a subset of T cells (Th17（英语：Th17）) seems to be important.[14] The primary cause is not well known. In fact, no one knows yet why the immune system starts to behave this way in Behçet's disease. There does however seem to be a genetic component involved, as first degree relatives of the affected patients are often affected in more than the expected proportion for the general population.[来源请求]

Research suggests that previous infections may provoke the autoimmune responses present in Behçet's disease. Heat shock proteins (HSPs) are present in some bacteria and serve as a "danger signal" to the immune system. However, some HSPs share a similarity in bacteria and humans.[15] The anti-HSP60（英语：HSP60） and anti-HSP65 antibodies that target HSPs produced by Streptococci (including S. sanguinis and S. pyogenes) and Mycobacterium tuberculosis can also target human HSPs, leading to immune responses linked to uveitis and various symptoms shown in parenchymal neuro-Behçet's disease.[16]

An association with the GIMAP ("GTPase（英语：GTPase） of the immunity-associated protein") family of genes on the long arm of chromosome 7 (7q36.1) has been reported.[17] Gene locations of single-nucleotide polymorphisms associated with Behçet's disease included GIMAP1, GIMAP2 and GIMAP4（英语：GIMAP4）.[17]

Pathophysiology

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HLA-B51（英语：HLA-B51） is strongly associated with Behçet's disease[18]

Behçet's disease is considered more prevalent in the areas surrounding the old silk trading routes in the Middle East and in Central Asia. Thus, it is sometimes known as Silk Road disease. However, this disease is not restricted to people from these regions. A large number of serological studies show a linkage between the disease and HLA-B51（英语：HLA-B51）.[19] HLA-B51 is more frequently found from the Middle East to South Eastern Siberia, but the incidence of B51 in some studies was 3 fold higher than the normal population. However, B51 tends not to be found in disease when a certain SUMO4（英语：SUMO4） gene variant is involved,[20] and symptoms appear to be milder when HLA-B27 is present.[21] At the current time, a similar infectious origin has not yet been confirmed that leads to Behçet's disease, but certain strains of S. sanguinis has been found to have a homologous antigenicity.[22]

Vasculitis resulting in occlusion of the vessels supplying the optic nerve may be the cause of acute optic neuropathy and progressive optic atrophy in Behçet's disease. Histological evaluation in a reported case of acute optic neuropathy demonstrated substitution of the axonal portion of the optic nerve with fibrous astrocytes without retinal changes.[7] CNS involvement in Behçet's disease may lead to intracranial hypertension most commonly due to dural venous sinus thrombosis[23] and subsequent secondary optic atrophy.

Diagnosis

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There is no specific pathological testing or technique available for the diagnosis of the disease, although the International Study Group criteria for the disease are highly sensitive and specific, involving clinical criteria and a pathergy（英语：pathergy） test.[4][24] Behçet's disease has a high degree of resemblance to diseases that cause mucocutaneous lesions such as Herpes simplex labialis, and therefore clinical suspicion should be maintained until all the common causes of oral lesions are ruled out from the differential diagnosis.[来源请求]

Visual acuity, or color vision loss with concurrent mucocutaneous lesions or systemic Behçet's disease symptoms should raise suspicion of optic nerve involvement in Behçet's disease and prompt a work-up for Behçet's disease if not previously diagnosed in addition to an ocular work-up. Diagnosis of Behçet's disease is based on clinical findings including oral and genital ulcers, skin lesions such as erythema nodosum, acne, or folliculitis, ocular inflammatory findings and a pathergy reaction. Inflammatory markers such ESR, and CRP may be elevated. A complete ophthalmic examination may include a slit lamp examination, optical coherence tomography to detect nerve loss, visual field examinations, fundoscopic examination to assess optic disc atrophy and retinal disease, fundoscopic angiography, and visual evoked potentials, which may demonstrate increased latency. Optic nerve enhancement may be identified on Magnetic Resonance Imaging (MRI) in some patients with acute optic neuropathy. However, a normal study does not rule out optic neuropathy. Cerebrospinal fluid (CSF) analysis may demonstrate elevated protein level with or without pleocytosis. Imaging including angiography may be indicated to identify dural venous sinus thrombosis as a cause of intracranial hypertension and optic atrophy.[来源请求]

Diagnostic guidelines

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Magnetic resonance venogram demonstrating occlusion of the left sigmoid and transverse sinuses

According to the International Study Group guidelines, for a patient to be diagnosed with Behçet's disease,[24] the patient must have oral (aphthous) ulcers (any shape, size, or number at least three times in any twelve-month period) along with two of the following four hallmark symptoms:[来源请求]

Despite the inclusive criteria set forth by the International Study Group, there are cases where not all the criteria can be met and therefore a diagnosis cannot readily be made. There is, however, a set of clinical findings that a physician can rely upon in making a tentative diagnosis of the disease; essentially, Behçet's disease does not always follow the International Study Group guidelines and so a high degree of suspicion for a patient who presents having any number of the following findings is necessary:[来源请求]

Treatment

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Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. The quality of the evidence for treating the oral ulcers associated with Behçet's disease, however, is poor.[25]

High-dose corticosteroid therapy is often used for severe disease manifestations.[26] Anti-TNF（英语：TNF inhibitor） therapy such as infliximab has shown promise in treating the uveitis associated with the disease.[27][28] Infliximab as well as other anti-TNF therapies including etanercept and adalimumab may be useful in treating mucocutaneous（英语：mucocutaneous） disease according to several case reports and prospective studies, as well as one randomized trial for etanercept.[29][30][31] Apremilast（英语：Apremilast） may also be used to treat oral ulcers associated with Behçet's disease.[32]

Interferon alpha-2a may also be an effective alternative treatment, particularly for the genital and oral ulcers[33] as well as ocular lesions.[34] Azathioprine, when used in combination with interferon alpha-2b also shows promise,[35] and colchicine can be useful for treating some genital ulcers, erythema nodosum, and arthritis.[36] Benzathine-penicillin may also reduce new arthritic attacks.[37]

Thalidomide has also been used due to its immune-modifying effect.[38] Dapsone and rebamipide（英语：rebamipide） have been shown, in small studies, to have beneficial results for mucocutaneous lesions.[39][40]

Given its rarity, the optimal treatment for acute optic neuropathy in Behçet's disease has not been established. Early identification and treatment are essential. Response to ciclosporin, periocular triamcinolone, and IV methylprednisolone followed by oral prednisone has been reported although relapses leading to irreversible visual loss may occur even with treatment.[41] Immunosuppressants such as interferon-alpha and tumour necrosis factor antagonists may improve though not completely reverse symptoms of ocular Behçet's disease, which may progress over time despite treatment. When symptoms are limited to the anterior chamber of the eye prognosis is improved. Posterior involvement, particularly optic nerve involvement, is a poor prognostic indicator. Secondary optic nerve atrophy is frequently irreversible. Lumbar puncture or surgical treatment may be required to prevent optic atrophy in cases of intracranial hypertension refractory to treatment with immunomodulators and steroids.[来源请求]

Intravenous immunoglobulin therapy (IVIg) could be a treatment for severe[42] or complicated cases.[43][44]

A recent 2024 reports that infliximab improved the likelihood of achieving a complete response at 22 weeks for patients with severe Behçet’s syndrome compared to cyclophosphamide, according to head-to-head trial data.[45] Mild to moderate adverse events, primarily infections, were reported in 29.6% of patients on infliximab and 64% on cyclophosphamide. Serious adverse events occurred in 15% and 12% of patients, respectively. [46]

Surgery

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Surgical treatment of arterial manifestations of BD bears many pitfalls since the obliterative endarteritis of vasa vasorum causes thickening of the medial layer and splitting of elastin fibers. Therefore, anastomotic pseudoaneurysms are likely to form, as well as pseudoaneurysms at the site of the puncture in case of angiography or endovascular treatment; furthermore, early graft occlusion may occur.[来源请求]

For these reasons, invasive treatment should not be performed in the acute and active phases of the disease when inflammation is at its peak. The evaluation of disease's activity is usually based on relapsing symptoms, ESR (erythrocyte sedimentation rate), and serum levels of CRP (C-reactive protein).[来源请求]

Endovascular treatment can be an effective and safe alternative to open surgery, with less postoperative complications, faster recovery time, and reduced need for intensive care, while offering patency rates and procedural success rates comparable with those of surgery. This notwithstanding, long-term results of endovascular treatment in BD are still to be determined.[来源请求]

Epidemiology

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The syndrome is rare in the United States, Africa and South America, but is common in Asia, suggesting a possible cause endemic to those areas.[47] A theory suggested that past exposure to lethal infectious agents might have fixed the genetic susceptibility factors to Behçet's disease in those area.[48] An estimated 15,000 to 20,000 Americans have been diagnosed with this disease. In the UK, it is estimated to have about 1 case for every 100,000 people.[49]

In an epidemiologic study, 56 percent of patients with Behçet's disease developed ocular involvement at a mean age of 30.[50] Ocular involvement was the first manifestation of Behçet's disease in 8.6 percent of patients.[50] Ocular Behçet's disease with involvement of the optic nerve is rarely reported. Among patients with ocular Behçet's disease funduscopic findings of optic atrophy, and optic disc paleness have been identified with a frequency of 17.9 percent and 7.4 percent, respectively. Other fundoscopic findings include vascular sheathing (23.7%),[6] retinal hemorrhage (9%),[6] macular edema (11.3%),[6] branch retinal vein occlusion (5.8%),[6] and retinal edema (6.6%).[6] However, optic atrophy was the most significant cause of visual impairment identified in 54 percent of patients with ocular Behçet's disease and permanent visual impairment.[6]

Pregnancy

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With Behçet's disease as a pre-existing disease in pregnancy（英语：pre-existing disease in pregnancy） or acquired, the pregnancy does not have an adverse effect on the course of Behçet's disease and may possibly ameliorate its course.[51][52] Still, there is a substantial variability in clinical course between patients and even for different pregnancies in the same patient.[51] Also, the other way around, Behçet's disease confers an increased risk of pregnancy complications, miscarriage and Cesarean section.[52]

Behçet's can cause infertility, either as a result of the condition itself or of a side effect of concomitant medication such as colchicine, which is known to lower sperm count.[53]

History

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The first modern formal description of the symptoms was made by H. Planner and F. Remenovsky and published in 1922 in the Archiv für Dermatologie und Syphilis（英语：Archiv für Dermatologie und Syphilis）.[54] Behçet's disease is named after Hulusi Behçet（英语：Hulusi Behçet） (1889–1948), the Turkish dermatologist and scientist who first recognized the three main symptoms of the syndrome in one of his patients in 1924 and reported his research on the disease in Journal of Skin and Venereal Diseases in 1936.[54][55] The name (Morbus Behçet) was formally adopted at the International Congress of Dermatology in Geneva in September 1947. Symptoms of this disease may have been described by Hippocrates in the 5th century BC, in his Epidemion (book 3, case 7).[56]

Some sources use the term "Adamantiades's syndrome" or "Adamantiades–Behçet syndrome", for the work done by Benediktos Adamantiades（英语：Benediktos Adamantiades）.[57] However, the current World Health Organization/ICD-10 standard is "Behçet's disease". In 1991, Saudi Arabian medical researchers described neuro-Behçet's disease（英语：neuro-Behçet's disease）,[58] a neurological involvement in Behçet's disease, considered one of the most devastating manifestations of the disease.[59] The mechanism can be immune-mediated or thrombotic.[60] The term dates back to at least 1990.[61]

References

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  1. ^ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 Fleming, Ray. Fast Facts About Behçet's Disease. www.niams.nih.gov. November 2014 [29 May 2017]. （原始内容存档于13 May 2017） （英语）. 
  2. ^ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 Behçet's Syndrome. NORD (National Organization for Rare Disorders). 2015 [29 May 2017]. （原始内容存档于11 February 2017）.  已忽略未知参数|df= (帮助)
  3. ^ Ball, Gene V.; Fessler, Barri J.; Bridges, S. Louis Jr. Oxford Textbook of Vasculitis 3. OUP Oxford. 2014: 491. ISBN 9780191667022. （原始内容存档于10 September 2017） （英语）.  已忽略未知参数|df= (帮助)
  4. ^ 4.00 4.01 4.02 4.03 4.04 4.05 4.06 4.07 4.08 4.09 Bolster MB. MKSAP 15 Medical Knowledge Self-assessment Program: Rheumatology. Philadelphia: American College of Physicians. 2009. ISBN 978-1-934465-30-1. 
  5. ^ Eye. Access : A case of anterior ischemic optic neuropathy associated with Behçet's disease : Eye. Eye (Nature.com). 7 January 2011, 25 (3): 395–396. PMC 3178306 可免费查阅. PMID 21212799. doi:10.1038/eye.2010.208. 
  6. ^ 6.0 6.1 6.2 6.3 6.4 6.5 6.6 Ozdal PC, Ortaç S, Taşkintuna I, Firat E. Posterior segment involvement in ocular Behçet's disease. Eur J Ophthalmol. 2002, 12 (5): 424–31. PMID 12474927. S2CID 13428859. doi:10.1177/112067210201200514. 
  7. ^ 7.0 7.1 Kansu T, Kirkali P, Kansu E, Zileli T. Optic neuropathy in Behçet's disease. J Clin Neuroophthalmol. December 1989, 9 (4): 277–80. PMID 2531168. 
  8. ^ Behçet syndrome. Yazıcı, Yusuf., Hatemi, Gulen., Seyahi, Emire., Yazici, Hasan. 2nd. Cham: Springer. 2020: 77. ISBN 978-3-030-24131-5. OCLC 1127393738. 
  9. ^ Schonberg, Stacy; Stokkermans, Thomas J. Episcleritis. StatPearls Publishing. 2023-08-07 [2024-07-02]. PMID 30521217. 
  10. ^ Adam C. Ring. Steven S. Agabegi; Elizabeth Agabegi , 编. Step-up to medicine 3rd. Philadelphia: Wolters Kluwer/Lippincott Williams & Wilkins. 2012-08-23: 266. ISBN 978-1-60913-360-3. 
  11. ^ Schiff, Steven; Moffatt, Roy; Mandel, William J.; Rubin, Stanley A. Acute myocardial infarction and recurrent ventricular arrhythmias in Behcet's syndrome需要付费订阅. American Heart Journal. 1982, 103 (3): 438–440. PMID 7064781. doi:10.1016/0002-8703(82)90289-7. 
  12. ^ ElGuindy MS, ElGuindy AM. Aneurysmal coronary artery disease: An overview. Global Cardiology Science and Practice (Glob Cardiol Sci Pract.). October 2017, 2017 (3): e201726. PMC 5856968 可免费查阅. PMID 29564347. doi:10.21542/gcsp.2017.26 可免费查阅. 
  13. ^ Behcet Disease: Overview – eMedicine Dermatology. [28 March 2009]. （原始内容存档于16 February 2009）.  已忽略未知参数|df= (帮助)
  14. ^ Hatemi G, Seyahi E, Fresko I, Hamuryudan V (2012). "Behçet's syndrome: a critical digest of the recent literature". Clin Exp Rheumatol
  15. ^ Direskeneli, H. Innate and Adaptive Responses to Heat Shock Proteins in Behcet's Disease. Genetics Research International. 2013, 2013: 249157. ISSN 2090-3154. PMC 3893747 可免费查阅. PMID 24490075. doi:10.1155/2013/249157 可免费查阅. 
  16. ^ Tanaka, T.; Yamakawa, N.; Koike, N.; Suzuki, J.; Mizuno, F.; Usui, M. Behçet's disease and antibody titers to various heat-shock protein 60s. Ocular Immunology and Inflammation. June 1999, 7 (2): 69–74. ISSN 0927-3948. PMID 10420201. doi:10.1076/ocii.7.2.69.4018. 
  17. ^ 17.0 17.1 Lee YJ, Horie Y, Wallace GR, Choi YS, Park JA, Choi JY, Song R, Kang YM, Kang SW, Baek HJ, Kitaichi N, Meguro A, Mizuki N, Namba K, Ishida S, Kim J, Niemczyk E, Lee EY, Song YW, Ohno S, Lee EB. Genome-wide association study identifies GIMAP as a novel susceptibility locus for Behcet's disease.. Annals of the Rheumatic Diseases. 1 September 2013, 72 (9): 1510–6. PMID 23041938. S2CID 206848143. doi:10.1136/annrheumdis-2011-200288. 
  18. ^ Ohno S, Ohguchi M, Hirose S, Matsuda H, Wakisaka A, Aizawa M. Close association of HLA-Bw51 with Behçet's disease. Arch. Ophthalmol. September 1982, 100 (9): 1455–8. PMID 6956266. doi:10.1001/archopht.1982.01030040433013. 
  19. ^ Durrani K, Papaliodis GN. The genetics of Adamantiades–Behcet's disease. Semin Ophthalmol. 2008, 23 (1): 73–9. PMID 18214795. S2CID 11695492. doi:10.1080/08820530701745264. 
  20. ^ Hou S, Yang P, Du L, Zhou H, Lin X, Liu X, Kijlstra A. SUMO4 gene polymorphisms in Chinese Han patients with Behcet's disease. Clin. Immunol. October 2008, 129 (1): 170–5. PMID 18657476. doi:10.1016/j.clim.2008.06.006. 
  21. ^ Ahn JK, Park YG. Human leukocyte antigen B27 and B51 double-positive Behçet uveitis. Arch. Ophthalmol. October 2007, 125 (10): 1375–80. PMID 17923546. doi:10.1001/archopht.125.10.1375 可免费查阅. 
  22. ^ Yanagihori H, Oyama N, Nakamura K, Mizuki N, Oguma K, Kaneko F. Role of IL-12B promoter polymorphism in Adamantiades-Behcet's disease susceptibility: An involvement of Th1 immunoreactivity against Streptococcus Sanguinis antigen. J. Invest. Dermatol. July 2006, 126 (7): 1534–40. PMID 16514412. doi:10.1038/sj.jid.5700203 可免费查阅. 
  23. ^ Fujikado T, Imagawa K. Dural sinus thrombosis in Behçet's disease – a case report. Jpn. J. Ophthalmol. 1994, 38 (4): 411–16. PMID 7723211. 
  24. ^ 24.0 24.1 International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. Lancet. May 1990, 335 (8697): 1078–80. PMID 1970380. S2CID 53261546. doi:10.1016/0140-6736(90)92643-V. 
  25. ^ Taylor J, Glenny AM, Walsh T, Brocklehurst P, Riley P, Gorodkin R, Pemberton MN. Taylor J , 编. Interventions for the management of oral ulcers in Behçet's disease. The Cochrane Database of Systematic Reviews. 25 September 2014, 9 (9): CD011018. PMC 6872426 可免费查阅. PMID 25254615. doi:10.1002/14651858.CD011018.pub2. 
  26. ^ CMDT (Current Medical Diagnosis & Treatment) 2007, Chapter 20, page 872
  27. ^ Sfikakis PP, Theodossiadis PG, Katsiari CG, Kaklamanis P, Markomichelakis NN. Effect of infliximab on sight-threatening panuveitis in Behcet's disease. Lancet. 2001, 358 (9278): 295–6. PMID 11498218. S2CID 23347314. doi:10.1016/S0140-6736(01)05497-6. 
  28. ^ Sfikakis PP. Behçet's disease: a new target for anti-tumour necrosis factor treatment. Ann Rheum Dis. 2002, 61 (Suppl 2): ii51–3. PMC 1766720 可免费查阅. PMID 12379622. doi:10.1136/ard.61.suppl_2.ii51. 
  29. ^ Melikoglu M, Fresko I, Mat C, Ozyazgan Y, Gogus F, Yurdakul S, Hamuryudan V, Yazici H. Short-term trial of etanercept in Behcet's disease: a double blind, placebo controlled study. J Rheumatol. 2005, 32 (1): 98–105. PMID 15630733. 
  30. ^ Arida, Aikaterini; Fragiadaki, Kalliopi; Giavri, Eirini; Sfikakis, Petros P. Anti-TNF Agents for Behçet's Disease: Analysis of Published Data on 369 Patients需要付费订阅. Seminars in Arthritis and Rheumatism. 2011-08-01, 41 (1): 61–70. ISSN 0049-0172. PMID 21168186. doi:10.1016/j.semarthrit.2010.09.002. 
  31. ^ Vallet, H.; Riviere, S.; Sanna, A.; Deroux, A.; Moulis, G.; Addimanda, O.; Salvarani, C.; Lambert, M.; Bielefeld, P.; Seve, P.; Sibilia, J.; Pasquali, Jl; Fraison, Jb; Marie, I.; Perard, L. Efficacy of anti-TNF alpha in severe and/or refractory Behçet's disease: Multicenter study of 124 patients. Journal of Autoimmunity. 2015-08-01, 62: 67–74. ISSN 0896-8411. PMID 26162757. doi:10.1016/j.jaut.2015.06.005. hdl:11380/1079761 可免费查阅. 
  32. ^ Search Orphan Drug Designations and Approvals. www.accessdata.fda.gov. [19 November 2019]. （原始内容存档于27 November 2020）. 
  33. ^ Alpsoy E, Durusoy C, Yilmaz E, Ozgurel Y, Ermis O, Yazar S, Basaran E. Interferon alfa-2a in the treatment of Behcet disease: a randomized placebo-controlled and double-blind study. Arch Dermatol. 2002, 138 (4): 467–71. PMID 11939808. doi:10.1001/archderm.138.4.467 可免费查阅. 
  34. ^ Kötter I, Zierhut M, Eckstein AK, Vonthein R, Ness T, Günaydin I, Grimbacher B, Blaschke S, Meyer-Riemann W, Peter HH, Stübiger N. Human recombinant interferon alpha-2a for the treatment of Behçet's disease with sight-threatening posterior or panuveitis. Br J Ophthalmol. 2003, 87 (4): 423–31. PMC 1771623 可免费查阅. PMID 12642304. doi:10.1136/bjo.87.4.423. 
  35. ^ Hamuryudan V, Ozyazgan Y, Fresko Y, Mat C, Yurdakul S, Yazici H. Interferon alpha combined with azathioprine for the uveitis of Behcet's disease: an open study. Isr Med Assoc J. 2002, 4 (11 Suppl): 928–30. PMID 12455182. 
  36. ^ Yurdakul S, Mat C, Tüzün Y, Ozyazgan Y, Hamuryudan V, Uysal O, Senocak M, Yazici H. A double-blind trial of colchicine in Behcet's syndrome. Arthritis Rheum. 2001, 44 (11): 2686–92. PMID 11710724. S2CID 19523919. doi:10.1002/1529-0131(200111)44:11<2686::AID-ART448>3.0.CO;2-H 可免费查阅. 
  37. ^ Saenz, Antonio; Ausejo, Monica; Shea, Beverley; Wells, George A; Welch, Vivian; Tugwell, Peter. Pharmacotherapy for Behcet's syndrome. Cochrane Database of Systematic Reviews. 1998-04-27, 1998 (2): CD001084. ISSN 1465-1858. PMC 7032645 可免费查阅. PMID 10796413. doi:10.1002/14651858.cd001084. 
  38. ^ Hamuryudan V, Mat C, Saip S, Ozyazgan Y, Siva A, Yurdakul S, Zwingenberger K, Yazici H. Thalidomide in the treatment of the mucocutaneous lesions of the Behcet syndrome. A randomized, double-blind, placebo-controlled trial. Ann Intern Med. 1998, 128 (6): 443–450. PMID 9499327. S2CID 12089634. doi:10.7326/0003-4819-128-6-199803150-00004. 
  39. ^ Matsuda T, Ohno S, Hirohata S, Miyanaga Y, Ujihara H, Inaba G, Nakamura S, Tanaka S, Kogure M, Mizushima Y. Efficacy of rebamipide as adjunctive therapy in the treatment of recurrent oral aphthous ulcers in patients with Behcet's disease: a randomised, double-blind, placebo-controlled study. Drugs in R&D. 2003, 4 (1): 19–28. PMID 12568631. S2CID 23711352. doi:10.2165/00126839-200304010-00002. 
  40. ^ Sharquie KE, Najim RA, Abu-Raghif AR. Dapsone in Behcet's disease: a double-blind, placebo-controlled, cross-over study. The Journal of Dermatology. 2002, 29 (5): 267–279. PMID 12081158. S2CID 23356719. doi:10.1111/j.1346-8138.2002.tb00263.x. 
  41. ^ Voros GM, Sandhu SS, Pandit R. Acute optic neuropathy in patients with Behçet's disease. Report of two cases. Ophthalmologica. 2006, 220 (6): 400–05. PMID 17095888. S2CID 45084854. doi:10.1159/000095869. 
  42. ^ Seider N, Beiran I, Scharf J, Miller B. Intravenous immunoglobulin therapy for resistant ocular Behçet's disease. Br J Ophthalmol. November 2001, 85 (11): 1287–8. PMC 1723778 可免费查阅. PMID 11673289. doi:10.1136/bjo.85.11.1287. 
  43. ^ Shutty B, Garg KJ, Swender D, Chernin L, Tcheurekdjian H, Hostoffer R. Optimal use of ivig in a patient with Behçet syndrome and common variable immunodeficiency. Ann. Allergy Asthma Immunol. July 2012, 109 (1): 84. PMID 22727170. doi:10.1016/j.anai.2012.05.014. 
  44. ^ Beales IL. Gastrointestinal involvement in Behçet's syndrome. Am. J. Gastroenterol. December 1998, 93 (12): 2633. PMID 9860455. S2CID 26407172. doi:10.1111/j.1572-0241.1998.02633.x. 
  45. ^ Infliximab bests cyclophosphamide as first-line treatment of severe Behçet's syndrome. www.healio.com. [2024-11-18] （英语）. 
  46. ^ Saadoun, David; Maalouf, Georgina; Vieira, Matheus; Trad, Salim; Lazaro, Estibaliz; Sacre, Karim; Plessier, Aurelie; Sené, Thomas; Koné-Paut, Isabelle; Noel, Nicolas; Mekinian, Arsène; Lambert, Marc; Ribeiro, Emmanuel; Mirault, Tristan; Mele, Nicolas. Infliximab versus Cyclophosphamide for Severe Behçet's Syndrome需要付费订阅. NEJM Evidence. 2024-10-22, 3 (11): EVIDoa2300354. ISSN 2766-5526. PMID 39437137. doi:10.1056/EVIDoa2300354 （英语）. 
  47. ^ Behcet's Syndrome: MedlinePlus. nih.gov. [19 September 2016]. （原始内容存档于4 July 2016）.  已忽略未知参数|df= (帮助)
  48. ^ Piga, M; Mathieu, A. The origin of Behçet's disease geoepidemiology: possible role of a dual microbial-driven genetic selection.. Clinical and Experimental Rheumatology. 2014, 32 (4 Suppl 84): S123–9. PMID 24447390. 
  49. ^ Choices, N. H. S. Behçet's disease – NHS Choices. www.nhs.uk. [19 September 2016]. （原始内容存档于3 March 2016）.  已忽略未知参数|df= (帮助)
  50. ^ 50.0 50.1 Krause L, Köhler AK, Altenburg A, Papoutsis N, Zouboulis CC, Pleyer U, Stroux A, Foerster MH. Ocular involvement in Adamantiades-Behçet's disease in Berlin, Germany. Graefes Arch. Clin. Exp. Ophthalmol. May 2009, 247 (5): 661–6. PMID 18982344. S2CID 23191896. doi:10.1007/s00417-008-0983-4. 
  51. ^ 51.0 51.1 Uzun S, Alpsoy E, Durdu M, Akman A. The clinical course of Behçet's disease in pregnancy: A retrospective analysis and review of the literature. The Journal of Dermatology. 2003, 30 (7): 499–502. PMID 12928538. S2CID 12860697. doi:10.1111/j.1346-8138.2003.tb00423.x. 
  52. ^ 52.0 52.1 Jadaon J, Shushan A, Ezra Y, Sela HY, Ozcan C, Rojansky N. Behcet's disease and pregnancy. Acta Obstetricia et Gynecologica Scandinavica. 2005, 84 (10): 939–944. PMID 16167908. S2CID 22363654. doi:10.1111/j.0001-6349.2005.00761.x 可免费查阅. 
  53. ^ Behçet's disease – Treatment 互联网档案馆的存档，存档日期5 April 2014. from National Health Service. Page last reviewed: 22 August 2012
  54. ^ 54.0 54.1 synd/1863 - Who Named It?
  55. ^ Behçet H. Über rezidivierende, aphtöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien. Dermatologische Wochenschrift, Hamburg. 1937, 105 (36): 1152–1163.  Reproduced in Viggor SF, Willis AM, Jawad AS. Behçet's disease. Grand Rounds. 2011, 11: L1–L2. doi:10.1102/1470-5206.2011.L001. （原始内容需要付费订阅存档于27 January 2012）.  已忽略未知参数|df= (帮助)
  56. ^ Verity DH, Wallace GR, Vaughan RW, Stanford MR. Behçet's disease: from Hippocrates to the third millennium. Br J Ophthalmol. 29 April 2003, 87 (9): 1175–83. PMC 1771837 可免费查阅. PMID 12928293. doi:10.1136/bjo.87.9.1175. 
  57. ^ B. Adamandiades. Sur un cas d'iritis à hypopyon récidivant. Annales d'oculistique（英语：Annales d'oculistique）, Paris, 1931, 168: 271–278.
  58. ^ Malhotra Ravi. Saudi Arabia. Practical Neurology. 2004, 4 (3): 184–185. doi:10.1111/j.1474-7766.2004.03-225.x (不活跃 2 January 2025). 
  59. ^ Saleem S. Neuro-Behçet's Disease: NBD. Neurographics. 2005, 4 (2): 1. （原始内容存档于16 March 2008）.  已忽略未知参数|df= (帮助)
  60. ^ Al-Araji A, Kidd DP. Neuro-Behçet's disease: epidemiology, clinical characteristics, and management. Lancet Neurol. February 2009, 8 (2): 192–204. PMID 19161910. S2CID 16483117. doi:10.1016/S1474-4422(09)70015-8. 
  61. ^ Su SL, Way LJ, Lin RT, Peng MJ, Wu SC. Neuro-Behçet's disease: report of three cases with a review of the literature. Gaoxiong Yi Xue Ke Xue Za Zhi. March 1990, 6 (3): 155–62. PMID 2188002. 

Further reading

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外部链接

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维基共享资源上的相关多媒体资源：贝赛特氏症
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