强直性脊柱炎

title: 症状和病理学.
content: 强直性脊柱炎主要的病程在于关节和腱的炎症,尤其在骨盆和脊柱部位,并伴随有水肿与骨髓的损伤,并导致骨髓的骨质化。  一般在23岁左右症状会逐渐显现,最初常见长期脊柱疼痛和僵硬,伴有旁侧臀部和大腿后部关节痛,其他原因也可能导致该疼痛,会影响人们对初期病情的判断。受累部位包括肌腱附着点以及关节囊。在关节软骨下方会生长肉芽组织,通过淋巴细胞和巨噬细胞形成浸润。关节受损的边缘,首先会通过纤维软骨(Faserknorpel)所替代,随后骨质化,由此关节逐渐僵硬化。在脊椎,此过程会导致骨骼出现韧带骨赘,并会搭上临近的脊椎骨。这样的过程最后可能导致所谓的竹节样脊柱改变(Bambuswirbelsaeule)的产生。  40%的患者会患虹膜炎,造成眼部发红、眼痛、失明、怕光。这是HLA-B27抗体在脊柱和虹膜都发生作用的结果。其他并发症还有胸痛、疲劳。  18岁前发病常导致四肢关节——尤其是膝关节——肿大疼痛。青春期前发病可能会踝关节和脚部关节肿痛,出现跟骨骨刺。
title: 起因.
content: 强直性脊柱炎的起因,医学上迄今未能完全了解,但该病似与免疫系统的缺陷有关。现代医学认为,如果以医学方法阻止TNF-α因子发挥作用,该病的症状得以减轻,可以得知该因子在发病过程中起着重要作用。在发炎的骶髂关节中可见CD4+T-淋巴细胞,细胞毒素T细胞(cytotoxic T-cells)及噬菌细胞出现,此外TNF-α因子的集中率见增高,不过无法确定触发此现象的触媒。可能是针对体内软骨中负责伸展灵活性的Proteoglycan Aggrecan产生自免疫现象(autoimmunologiesches Phenomenon)。这样Proteoglycan抗原的一致性,可以解释疾病在体内分布的现象。在许多患者的血液中还出现了肠内细菌(Enterobakterien)抗体滴定浓度增高的现象,目前尚未弄清此现象与该病有何关联。  该病的一个特征是,发病与血液中HLA-B27因子的高度关联性。这种关联性在不同人种中出现的频率揭示该病在不同人种中发病率的不同。时至今日已比较清楚的是,强直性脊柱炎很大程度上是由基因决定的,因此HLA-B27因子是此病最为人所熟知,但绝不是唯一的基因病因。 2007年,Wellcome Trust病例控制协会(Wellcome Trust Case Control Consortium,WTCCC)和澳-英-美脊柱炎协会(Australo-Anglo-American Spondyloarthritis Consortium,TASC)进行的14500个非同义单核苷酸多态性研究(non-synonymous SNPs),还发现AS与ERAP-1和IL-23R两个基因的关联。另一项是TASC涉及的完整的全基因组关联的研究,进一步关联分析研究发现与基因沙漠中的2p15和21q22有关。
title: 常用的治疗法.
content: 主要分三方向
title: 消炎止痛.
content: 消炎痛等非甾体抗炎药(非类固醇消炎止痛药)解热镇痛药具有较好的效果。柳氮磺胺吡啶对于改善腰背部僵痛及骶髂关节病变有明显的疗效。
title: 控制病情.
content: 严重的周围关节病变也可采用氨甲喋呤 Methotrexate 治疗。当髋关节病变严重,造成骨性强直和瘫痪时,早期施行人工髋关节置换术,以恢复功能。
title: 保护关节-保持灵活.
content: 强直性脊柱炎需要长期治疗,甚至终身治疗,患者应对自己的疾病有正确的认识,积极配合医生的治疗。体疗的康复训练对于本病具有非常重要的意义。适当的行走、睡眠姿势、腰腹的锻练可以维持关节的灵活性,呼吸锻练和游泳的有氧运动有助于维持良好的肺功能。部分患者经过治疗后疗效不明显,出现关节僵直,但如果能够维持在功能位,就可最大限度地保留部分关节功能。
title: 性别与种族差异性.
content: 强直性脊柱炎多发于男性青少年,男女发病的比例约为2.5-5:1。此外,该病症与民族或种族差异也表现出一定关联,如黑人的发病率明显低于黄种人及白种人;而在东亚日本的发病率又明显低于中、韩等国。  在中国,该病的发病率约为千分之三。

Summary: 强直性脊柱炎(拉丁文:spondylitis ankylosans,其中spondylitis原为希腊文脊柱炎之意,ankylosans原系希腊文强直之意),又称僵直性脊椎炎,在欧陆亦称此病为白赫铁列夫症(Morbus Bechterew)、白赫铁列夫-史特林佩尔-玛丽症(Bechterew-Struempell-Marie-Krankheit)等。英文为ankylosing spondylitis,国际简称为AS,是一种长期脊椎关节有发炎症状的关节炎,通常脊椎连接骨盆的关节会受到影响,偶尔还会侵犯其他部位的关节,如肩膀或臀部,眼睛和肠道问题也可能发生。背痛是僵直性脊椎炎的特征,而背痛的情况往往是来来去去的。受影响的关节僵硬程度通常会随着时间推移而恶化。 尽管僵直性脊椎炎的成因尚不清楚,但被推测和遗传与环境之综合因素有关,超过90%的患者具有称为HLA-B27抗原的特异性人类白细胞抗原,,故基本机转认为是与自体免疫或自身炎症性有关。僵直性脊椎炎的确诊除了症状表现外,通常需辅以临床医学影像和血液检查的数据支持。僵直性脊椎炎是一种血清阴性脊椎关节病变,这意味着其不具有类风湿因子(RF)的抗体。僵直性脊椎炎也可被更广泛地分类为轴性的脊椎关节病变。 僵直性脊椎炎无法治愈,不过可以透过治疗改善症状,避免症状加剧,治疗方式包括药物、运动及手术。药物包括有非甾体抗炎药(NSAID)、皮质类固醇、像柳氮磺胺吡啶等缓解疾病抗风湿药物,以及infliximab等生物制剂。 罹患僵直性脊椎炎的比例约在0.1%至1.8%之间,多半容易在青年人身上发病,男性比女性容易患有僵直性脊椎炎。此症状最早是在十七世纪末由Bernard Connor完整描述,而在古埃及木乃伊中就发现有僵直性脊椎炎的骨骸。僵直性脊椎炎的英文Ankylosing spondylitis源自希腊文的ankylos(表示硬化)、spondylos(表示脊椎骨)及-itis(表示发炎)。

Ankylosing spondylitis: Ankylosing spondylitis (AS) is a type of arthritis in which there is a long-term inflammation of the joints of the spine. Typically the joints where the spine joins the pelvis are also affected. Occasionally other joints such as the shoulders or hips are involved. Eye and bowel problems may also occur. Back pain is a characteristic symptom of AS, and it often comes and goes. Stiffness of the affected joints generally worsens over time. Although the cause of ankylosing spondylitis is unknown, it is believed to involve a combination of genetic and environmental factors. More than 85% of those affected in the UK have a specific human leukocyte antigen known as the HLA-B27 antigen. The underlying mechanism is believed to be autoimmune or autoinflammatory. Diagnosis is typically based on the symptoms with support from medical imaging and blood tests. AS is a type of seronegative spondyloarthropathy, meaning that tests show no presence of rheumatoid factor (RF) antibodies. It is also within a broader category known as axial spondyloarthritis. There is no cure for ankylosing spondylitis. Treatments may improve symptoms and prevent worsening. This may include medication, exercise, physical therapy, surgery in rare cases. Medications used include NSAIDs, steroids, DMARDs such as sulfasalazine, and biologic agents such as TNF inhibitors. Between 0.1% and 1.8% of people are affected. Onset is typically in young adults. Males and females are equally affected. It used to be thought that three times as many men as women had the disease. This was based on a diagnosis of the disease using x-ray. Men are more likely than women to experience changes to the bones and fusion, and thus they were being picked up using x-ray. Over time MRI’s were developed which could identify inflammation. Women are more likely than men to experience inflammation rather than fusion. source The condition was first fully described in the late 1600s by Bernard Connor, but skeletons with ankylosing spondylitis are found in Egyptian mummies. The word is from Greek "ankylos" meaning to unite or grow together, "spondylos" meaning vertebra, and "-itis" meaning inflammation.