ankylosing spondylitis

強直性脊椎炎

WordNet

1. inflammation of a spinal joint; characterized by pain and stiffness

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/05/31 15:14:42」(JST)

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Ankylosing spondylitis
Synonyms	Bekhterev's disease, Bechterew's disease, morbus Bechterew, Bekhterev-Strümpell-Marie disease, Marie's disease, Marie–Strümpell arthritis, Pierre–Marie's disease
A 6th century skeleton showing fused vertebrae, a sign of severe ankylosing spondylitis.
Specialty	Rheumatology
Symptoms	Back pain, joint stiffness
Usual onset	Young adulthood
Duration	Long term
Causes	Unknown
Diagnostic method	Symptom based, medical imaging, blood tests
Treatment	medication, exercise, and surgery
Medication	NSAIDs, steroids, DMARDs
Frequency	0.1 to 1.8%

Ankylosing spondylitis (AS) is a type of arthritis in which there is 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.

The cause of ankylosing spondylitis is unknown; however, it is believed to involve a combination of genetic and environmental factors. More than 90% of those affected 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, and surgery. Medications used include NSAIDs, steroids, DMARDs such as sulfasalazine, and biologic agents such as infliximab.

Between 0.1% and 1.8% of people are affected. Onset is typically in young adults. Males are more often affected than females. The condition was first fully described in the late 1600s by Bernard Connor; however, skeletons with ankylosing spondylitis are found in Egyptian mummies. The word is from Greek ankylos meaning stiffening, spondylos meaning vertebra, and -itis meaning inflammation.

Signs and symptoms

Illustration depicting ankylosing spondylitis.

The signs and symptoms of ankylosing spondylitis often appear gradually, with peak onset being between 20 and 30 years of age.

The initial symptoms are usually a chronic dull pain in the lower back or gluteal region combined with stiffness of the lower back.

Individuals often experience pain and stiffness that awakens them in the early morning hours.

As the disease progresses, loss of spinal mobility and chest expansion, with a limitation of anterior flexion, lateral flexion, and extension of the lumbar spine, are seen.

Systemic features are common, with weight loss, fever, or fatigue often present.

Pain is often severe at rest but improves with physical activity. However, many experience inflammation and pain to varying degrees regardless of rest and movement.

AS can occur in any part of the spine or the entire spine, often with pain referred to one or the other buttock or the back of the thigh from the sacroiliac joint.

Arthritis in the hips and shoulders may also occur.

When the condition presents before the age of 18, it is more likely to cause pain and swelling of large lower limb joints, such as the knees. In prepubescent cases, pain and swelling may also manifest in the ankles and feet where heel pain and enthesopathy commonly develop. Less commonly ectasia of the sacral nerve root sheaths may occur.

About 40% of people with AS will also experience inflammation of the anterior chamber of the eye, causing eye pain, redness, floaters and sensitivity to light. This is thought to be due to the association that both AS and uveitis have with the inheritance of the HLA-B27 antigen. Inflammation of the prostate occurs with increased frequency in men. Cardiovascular involvement may include inflammation of the aorta, aortic valve insufficiency or disturbances of the heart's electrical conduction system. Lung involvement is characterized by progressive fibrosis of the upper portion of the lung.

Pathophysiology

The ankylosis process

Ankylosing spondylitis (AS) is a systemic rheumatic disease, meaning it affects the entire body. Approximately 90% of people with AS express the HLA-B27 genotype, meaning there is a strong genetic association. 1-2% of individuals with the HLA-B27 genotype contract the disease. Tumor necrosis factor-alpha (TNF α) and IL-1 are also implicated in ankylosing spondylitis. Autoantibodies specific for AS have not been identified. Anti-neutrophil cytoplasmic antibodies (ANCAs) are associated with AS, but do not correlate with disease severity.

Single nucleotide polymorphism (SNP) A/G variant rs10440635 close to the PTGER4 gene on human chromosome 5 has been associated with an increased incidence of Ankylosing spondylitis in a population recruited from the United Kingdom, Australia, and Canada. The PTGER4 gene codes for the prostaglandin EP4 receptor (EP4), one of four receptors for prostaglandin E2. Activation of EP4 promotes bone remodeling and deposition (see EP4, bone) and EP4 is highly expressed at vertebral column sites involved in ankylosing spondylitis. These findings suggest that excessive EP4 activation contributes to pathological bone remodeling and deposition in ankylosing spondylitis and that the A/G variant rs10440635a of PTGER4 predisposes to this disease, possibly by influencing EP4's production or expression pattern.

The association of AS with HLA-B27 suggests the condition involves CD8 T cells, which interact with HLA-B. This interaction is not proven to involve a self-antigen, and at least in the related reactive arthritis, which follows infections, the antigens involved are likely to be derived from intracellular microorganisms. There is, however, a possibility that CD4+ T lymphocytes are involved in an aberrant way, since HLA-B27 appears to have a number of unusual properties, including possibly an ability to interact with T cell receptors in association with CD4 (usually CD8+ cytotoxic T cell with HLAB antigen as it is a MHC class 1 antigen).

"Bamboo spine" develops when the outer fibers of the fibrous ring (annulus fibrosus disci intervertebralis) of the intervertebral discs ossify, which results in the formation of marginal syndesmophytes between adjoining vertebrae.

Diagnosis

34-year-old male with AS. Inflammatory lesions of the anterior chest wall are shown (curved arrows). Inflammatory changes are seen in the lower thoracic spine and L1 (arrows).

Ankylosing spondylitis is a member of the more broadly defined disease axial spondyloarthritis. Axial spondyloarthritis can be divided into (1) radiographic axial spondyloarthritis (which is a synonym for ankylosing spondylitis) and (2) non-radiographic axial spondyloarthritis (which include less severe forms and early stages of ankylosing spondylitis)

While ankylosing spondylitis can be diagnosed through the description of radiological changes in the sacroiliac joints and spine, there are currently no direct tests (blood or imaging) to unambiguously diagnose early forms of ankylosing spondylitis (non-radiographic axial spondyloarthritis). Diagnosis of non-radiologic axial spondyloarthritis is therefore more difficult and is based on the presence of several typical disease features.

These diagnostic criteria include:

• Inflammatory back pain:
  Chronic, inflammatory back pain is defined when at least four out of five of the following parameters are present: (1) Age of onset below 40 years old, (2) insidious onset, (3) improvement with exercise, (4) no improvement with rest, and (5) pain at night (with improvement upon getting up)
• Past history of inflammation in the joints, heels, or tendon-bone attachments
• Family history for axial spondyloarthritis
• Positive for the biomarker HLA-B27
• Good response to treatment with nonsteroidal anti-inflammatory drugs (NSAIDs)
• Signs of elevated inflammation (C-reactive protein and erythrocyte sedimentation rate)
• Manifestation of psoriasis, inflammatory bowel disease, or inflammation of the eye (uveitis)

If these criteria still do not give a compelling diagnosis magnetic resonance imaging (MRI) may be useful. MRI can show inflammation of the sacroiliac joint.

Radiographic features

• The earliest changes in the sacroiliac joints demonstrable by plain x–ray shows erosions and sclerosis.
• Progression of the erosions leads to pseudo-widening of the joint space and bony ankylosis.
• X-ray spine can reveal squaring of vertebrae with spine ossification with fibrous band run longitudinally called syndesmophyte while producing bamboo spine appearance.
• A drawback of X-ray diagnosis is the signs and symptoms of AS have usually been established as long as 8–10 years prior to X-ray-evident changes occurring on a plain film X-ray, which means a delay of as long as 10 years before adequate therapies can be introduced. Options for earlier diagnosis are tomography and MRI of the sacroiliac joints, but the reliability of these tests is still unclear.

• Lateral X-ray of the mid back in ankylosing spondylitis

• Lateral X-ray of the neck in ankylosing spondylitis

• X-ray showing bamboo spine in a person with ankylosing spondylitis.

• CT scan showing Bamboo spine in ankylosing spondylitis

• T1-weighted MRI with fat suppression after administration of gadolinium contrast showing sacroiliitis in a patient with ankylosing spondylitis

Blood parameters

During acute inflammatory periods, people with AS may show an increase in the blood concentration of CRP and an increase in the ESR, but there are many with AS whose CRP and ESR rates do not increase, so normal CRP and ESR results do not always correspond with the amount of inflammation that is actually present. In other words, some people with AS have normal levels of CRP and ESR, despite experiencing a significant amount of inflammation in their bodies.

Genetic testing

Variations of the HLA-B gene increase the risk of developing ankylosing spondylitis, although it is not a diagnostic test. Those with the HLA-B27 variant are at a higher risk than the general population of developing the disorder. HLA-B27, demonstrated in a blood test, can occasionally help with diagnosis, but in itself is not diagnostic of AS in a person with back pain. Over 90% of people that have been diagnosed with AS are HLA-B27 positive, although this ratio varies from population to population (about 50% of African Americans with AS possess HLA-B27 in contrast to the figure of 80% among those with AS who are of Mediterranean descent).

BASDAI

The Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), developed in Bath (UK), is an index designed to detect the inflammatory burden of active disease. The BASDAI can help to establish a diagnosis of AS in the presence of other factors such as HLA-B27 positivity, persistent buttock pain which resolves with exercise, and X-ray or MRI-evident involvement of the sacroiliac joints. It can be easily calculated and accurately assesses the need for additional therapy; a person with AS with a score of four out of a possible 10 points while on adequate NSAID therapy is usually considered a good candidate for biologic therapy.

The Bath Ankylosing Spondylitis Functional Index (BASFI) is a functional index which can accurately assess functional impairment due to the disease, as well as improvements following therapy. The BASFI is not usually used as a diagnostic tool, but rather as a tool to establish a current baseline and subsequent response to therapy.

Children

Juvenile anyklosing spondylitis (JAS) is a rare form of the disease which differs from the more common adult form. Enthesophathy and arthritis of large joints of the lower extremities is more common than the characteristic early-morning backpain seen in adult AS. Ankylosing tarsitis of the ankle is a common feature, as is the more classical findings of seronegative ANA and RF as well as presence of the HLA-B27 allele. Primary engagement of the appendicular joints may explain delayed diagnosis, however other common symptoms of AS such as uveitis, diarrhoea, pulmonary disease and heart valve disease may lead suspicion away from other juvenile spondyloarthropathies.

Schober's test

The Schober's test is a useful clinical measure of flexion of the lumbar spine performed during the physical examination.

Treatment

There is no cure for AS, although treatments and medications can reduce symptoms and pain.

Medication

The major types of medications used to treat ankylosing spondylitis are pain-relievers and drugs aimed at stopping or slowing the progression of the disease. All of these have potentially serious side effects. Pain-relieving drugs come in two major classes:

• The mainstay of therapy in all seronegative spondyloarthropathies are anti-inflammatory drugs, which include NSAIDs such as ibuprofen, phenylbutazone, diclofenac, indomethacin, naproxen and COX-2 inhibitors, which reduce inflammation and pain. Indomethacin is a drug of choice. 2012 research showed that those with AS and elevated levels of acute phase reactants seem to benefit most from continuous treatment with NSAIDs.
• Opioid painkillers

Medications used to treat the progression of the disease include the following:

• Disease-modifying antirheumatic drugs (DMARDs) such as sulfasalazine can be used in people with peripheral arthritis. For axial involvement, evidence does not support sulfasalazine. Other DMARDS, such as methotrexate, did not have enough evidence to prove their effect. Generally, systemic corticosteroids were not used due to lack of evidence. Local injection with corticosteroid can be used for certain people with peripheral arthritis.
• Tumor necrosis factor-alpha (TNFα) blockers (antagonists), such as the biologics etanercept, infliximab, golimumab and adalimumab, have shown good short-term effectiveness in the form of profound and sustained reduction in all clinical and laboratory measures of disease activity. Trials are ongoing to determine their long-term effectiveness and safety. The major drawback is the cost. An alternative may be the newer, orally-administered non-biologic apremilast, which inhibits TNF-α secretion, but a recent study did not find the drug useful for ankylosing spondylitis.
• Anti-interleukin-6 inhibitors such as tocilizumab, currently approved for the treatment of rheumatoid arthritis, and rituximab, a monoclonal antibody against CD20, are also undergoing trials.
• Interleukin-17A inhibitor secukinumab is an option for the treatment of active ankylosing spondylitis that has responded inadequately to (TNFα) blockers.

Surgery

In severe cases of AS, surgery can be an option in the form of joint replacements, particularly in the knees and hips. Surgical correction is also possible for those with severe flexion deformities (severe downward curvature) of the spine, particularly in the neck, although this procedure is considered very risky.

In addition, AS can have some manifestations which make anesthesia more complex. Changes in the upper airway can lead to difficulties in intubating the airway, spinal and epidural anesthesia may be difficult owing to calcification of ligaments, and a small number of people have aortic insufficiency. The stiffness of the thoracic ribs results in ventilation being mainly diaphragm-driven, so there may also be a decrease in pulmonary function.

Physical therapy

Though physical therapy remedies have been scarcely documented, some therapeutic exercises are used to help manage lower back, neck, knee, and shoulder pain. Some therapeutic exercises include:

• Low intensity aerobic exercise
• Transcutaneous electrical nerve stimulation (TENS)
• Thermotherapy
• Proprioceptive neuromuscular facilitation (PNF)
• Exercise programs, either at home or supervised;
• Group exercises;

Moderate-to-high impact exercises like jogging are generally not recommended or recommended with restrictions due to the jarring of affected vertebrae that can worsen pain and stiffness in some with AS.

Prognosis

Fracture of the T5 and C7 vertebra due to trauma in a person with ankylosing spondylitis as seen on a CT scan

Prognosis is related to disease severity. AS can range from mild to progressively debilitating and from medically controlled to refractory. Some cases may have times of active inflammation followed by times of remission resulting in minimal disability while others never have times of remission and have acute inflammation and pain, leading to significant disability. As the disease progresses, it can cause the vertebrae and the lumbosacral joint to ossify, resulting in the fusion of the spine. This places the spine in a vulnerable state because it becomes one bone, which causes it to lose its range of motion as well as putting it at risk for spinal fractures. This not only limits mobility but reduces the affected person's quality of life. Complete fusion of the spine can lead to a reduced range of motion and increased pain, as well as total joint destruction which could lead to a joint replacement.

Osteoporosis is common in ankylosing spondylitis, both from chronic systemic inflammation and decreased mobility resulting from AS. Over a long-term period, osteopenia or osteoporosis of the AP spine may occur, causing eventual compression fractures and a back "hump". Hyperkyphosis from ankylosing spondylitis can also lead to impairment in mobility and balance, as well as impaired peripheral vision, which increases the risk of falls which can cause fracture of already-fragile vertebrae. Typical signs of progressed AS are the visible formation of syndesmophytes on X-rays and abnormal bone outgrowths similar to osteophytes affecting the spine. In compression fractures of the vertebrae, paresthesia is a complication due to the inflammation of the tissue surrounding nerves.

Organs commonly affected by AS, other than the axial spine and other joints, are the heart, lungs, eyes, colon, and kidneys. Other complications are aortic regurgitation, Achilles tendinitis, AV node block and amyloidosis. Owing to lung fibrosis, chest X-rays may show apical fibrosis, while pulmonary function testing may reveal a restrictive lung defect. Very rare complications involve neurologic conditions such as the cauda equina syndrome.

Mortality

Mortality is increased in people with AS and circulatory disease is the most frequent cause of death. AS patients have an increased risk of 60% for cerebrovascular mortality, and an overall increased risk of 50% for vascular mortality. About one third of those with Ankylosing spondylitis have severe disease, which reduces life expectancy.

As increased mortality in ankylosing spondylitis is related to disease severity, factors negatively affecting outcomes include:

• Male sex
• Plus 3 of the following in the first 2 years of disease:
  • Erythrocyte sedimentation rate (ESR) >30 mm/h
  • Unresponsive to NSAIDs
  • Limitation of lumbar spine range of motion
  • Sausage-like fingers or toes
  • Oligoarthritis
  • Onset <16 years old

Gait

The hunched position that often results from complete spinal fusion can have an effect on a person’s gait. Increased spinal kyphosis will lead to a forward and downward shift in center of mass (COM). This shift in COM has been shown to be compensated by increased knee flexion and ankle dorsiflexion. The gait of someone with ankylosing spondylitis often has a cautious pattern because they have decreased ability to absorb shock, and they cannot see the horizon.

Epidemiology

Ankylosing spondylitis prevalence is between 0.1 and 0.2% of the general population. The disease is most prevalent in Northern European countries, and seen least in people of Afro-Caribbean descent. The ratio of male to female disease is 3:1, however many rheumatologists believe the number of women with AS is underdiagnosed, as most women tend to experience milder cases of the disease. The majority of people with AS, including 95 percent of white people with the disease, express the HLA-B27 antigen and high levels of immunoglobulin A (IgA) in the blood.

Research

In 2007, a collaborative effort by an international team of researchers in the United Kingdom, Australia and the United States led to the discovery of two genes that also contribute to the cause of AS: ARTS-1 and IL23R. The findings were published in the November 2007 edition of Nature Genetics, a journal that emphasizes research on the genetic basis for common and complex diseases. Together with HLA-B27, these two genes account for roughly 70 percent of the overall incidence of the disease.

History

Drawing from 1857 shows a severe case of AS.

Ankylosing spondylitis has a long history, having been distinguished from rheumatoid arthritis by Galen as early as the 2nd century AD. Skeletal evidence of the disease (ossification of joints and entheses primarily of the axial skeleton, known as "bamboo spine") was found in an archaeological dig that unearthed the skeletal remains of a 5000-year-old Egyptian mummy with evidence of bamboo spine.

The anatomist and surgeon Realdo Colombo described what could have been the disease in 1559, and the first account of pathologic changes to the skeleton possibly associated with AS was published in 1691 by Bernard Connor. In 1818, Benjamin Brodie became the first physician to document a person believed to have active AS who also had accompanying iritis.

In 1858, David Tucker published a small booklet which clearly described the case of Leonard Trask, who suffered from severe spinal deformity subsequent to AS. In 1833, Trask fell from a horse, exacerbating the condition and resulting in severe deformity. Tucker reported:

It was not until he [Trask] had exercised for some time that he could perform any labor ... [H]is neck and back have continued to curve drawing his head downward on his breast.

This account became the first documented case of AS in the United States, owing to its indisputable description of inflammatory disease characteristics of AS and the hallmark of deforming injury in AS.

It was not until the late nineteenth century, however, when the neurophysiologist Vladimir Bekhterev of Russia in 1893, Adolph Strümpell of Germany in 1897, and Pierre Marie of France in 1898 were the first to give adequate descriptions which permitted an accurate diagnosis of AS prior to severe spinal deformity. For this reason, AS is also known as Bekhterev Disease, Bechterew's Disease or Marie–Strümpell Disease.

See also

• List of people with ankylosing spondylitis

References

External links

Classification	ICD-10: M08.1, M45 ICD-9-CM: 720.0 OMIM: 106300 MeSH: D013167 DiseasesDB: 728
External resources	MedlinePlus: 000420 eMedicine: radio/41 Patient UK: Ankylosing spondylitis

• Ankylosing spondylitis at DMOZ
• Ankylosing spondylitis - University of Washington Department of Orthopaedics & Sports Medicine Patient Article
• Questions and Answers about Ankylosing Spondylitis - US National Institute of Arthritis and Musculoskeletal and Skin Diseases

UpToDate Contents

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• 1. 成人における強直性脊椎炎の臨床症状 clinical manifestations of ankylosing spondylitis in adults
• 2. 成人における強直性脊椎炎の評価および治療 assessment and treatment of ankylosing spondylitis in adults
• 3. 成人における強直性脊椎炎およびX線所見による変化が認められない軸性脊椎関節炎の診断および鑑別診断 diagnosis and differential diagnosis of ankylosing spondylitis and non radiographic axial spondyloarthritis in adults
• 4. 強直性脊椎炎、末梢関節炎を主体とする脊椎関節炎、およびX線所見が認められない軸性脊椎関節炎における抗腫瘍壊死因子α（TNF-α）剤使用のための一般的ガイドライン general guidelines for use of anti tumor necrosis factor alpha agents in ankylosing spondylitis and in peripheral and non radiographic axial spondyloarthritis
• 5. 成人における選択的脊椎手術のための麻酔 anesthesia for elective spine surgery in adults

English Journal

• Meta-analysis reveals an association of PTPN22 C1858T with autoimmune diseases, which depends on the localization of the affected tissue.

• Zheng J, Ibrahim S, Petersen F, Yu X.SourceLaboratory of Autoimmunity, The Medical College of Xiamen University, Xiamen University, Xiamen, China.
• Genes and immunity.Genes Immun.2012 Oct 18. doi: 10.1038/gene.2012.46. [Epub ahead of print]
• Protein tyrosine phosphatase non-receptor type 22 (PTPN22) is a strong susceptibility gene shared by many autoimmune diseases. The aim of this study was to explore the mechanisms underlying this relationship. We performed a comprehensive analysis of the association between PTPN22 polymorphism C1858T
• PMID 23076337

• A systematic literature review of the economic impact of ankylosing spondylitis.

• Palla I, Trieste L, Tani C, Talarico R, Cortesi PA, Mosca M, Turchetti G.SourceIstituto di Management, Scuola Superiore Sant'Anna, Pisa, Italy. i.palla@sssup.it.
• Clinical and experimental rheumatology.Clin Exp Rheumatol.2012 Oct 16. [Epub ahead of print]
• This article reviews the last decade studies on the economic impact of ankylosing spondylitis (AS). Interestingly, a common observation is that in AS indirect costs are higher than the use of direct healthcare resources. Country, age, gender, and severity of the diseases impact on per patient annual
• PMID 23072824

Japanese Journal

• 長期間抗抗TNF-α製剤を投与した強直性脊椎炎の2例

• 整形外科と災害外科 66(1), 71-75, 2017
• NAID 130005632121

• The diagnostic dilemma of tumor induced osteomalacia: a retrospective analysis of 144 cases

• Endocrine Journal, 2017
• NAID 130005616292

• The immune dysfunction in ankylosing spondylitis patients

• BioScience Trends 11(1), 69-76, 2017
• NAID 130005466227

Related Links

• About Spondylitis

Ankylosing spondylitis, spondyloarthritis and related disease information, education and support. Ankylosing spondylitis and spondyloarthritis symptoms, diagnosis, treatment, medication, educational materials, brochures. Axial ...

• Overview - Ankylosing spondylitis - Mayo Clinic

Ankylosing spondylitis is an inflammatory disease that, over time, can cause some of the vertebrae in your spine to fuse. This fusing makes the spine less flexible and can result in a hunched-forward posture. If ribs are affected, it can ...

Related Pictures

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★リンクテーブル★

リンク元	「強直性脊椎炎」「ヒト白血球抗原」「rheumatoid spondylitis」「Bechterew's disease」「Marie-Strumpell disease」
関連記事	「ankylosing」

「強直性脊椎炎」

　　[★]

英

ankylosing spondylitis AS

同

リウマチ性脊椎炎、ベヒテレフ病、マリー-シュトリュンペル病 Marie-Struempell disease

関

血清反応陰性脊椎関節症

概念

• リウマトイド因子陰性で脊椎と仙腸関節が侵される疾患。

原因

• 原因は不明
• HLA-B27は95%前後陽性

疫学

• 発生頻度は0.04%程度で家族内発生が多い。
• 好発年齢：10歳台後半～20歳代台
• 男女比：90%男性。　男:女=9:1-5:1

病態

• 靭帯付着部などの関節辺縁に限局した骨炎が生じ、軟骨下骨や線維軟骨が肉芽組織に置換され、炎症が収まるのに伴いこの肉芽組織が骨化し、骨強直を来す。
• 踵骨部・坐骨結節に腱付着部症、仙腸関節である。

症状

• 腰痛、殿部や背部の痛み。
• 運動制限
• 深呼吸時の胸痛、関節痛、アキレス腱痛

合併症

• ぶどう膜炎
• 大動脈弁付近の炎症　→　強直性脊椎炎　＋　大動脈弁逆流症(AR)　？？？？

検査

血液検査

• リウマチ因子：陰性
• HLA-B27：90%の症例で陽性
• 赤沈：亢進

X線像

• 初期は仙腸関節部の両側性の辺縁不整。びらんが出現すると関節裂隙が開大して見える。次いで、びらんの周辺に硬化像が認められ、関節裂隙は狭小化し、最終的に硬直に至る(骨性強直)。
• 脊椎：前縦靭帯の椎体付着部からの骨化(椎体骨棘形成 syndesmophyte)　→　側面像での椎体の方形化 squaring　→　竹様脊柱 bamboo spine

診断

診断基準(東京都特殊疾病(難病)息者診断手引き, 1990年)

• I. 主要症状

• 1.腰痛(最低3カ月以上,運動で軽快し,安静による効果なし)
• 2.腰椎の可動制限(失状および前額面)

• a.前屈測定検査

後腸骨棟の高さで,垂直に測定した10cmの間隔が前屈で何cm伸延したかを計測：異常:5cm以下

• b.側屈測定検査

腋窩正中腺状,任意に引かれた20cmの線が側屈で何cm伸延したかを計測：異常:5cm以下

• 3.胸郭拡張の低下

胸郭拡張測定検査

第四肋間の高さで,最大呼気時の胸囲と最大吸気時の胸囲との差を計測：異常: 2.5cm以下

• II. 必要検査

• 1.仙腸関節X線像

• (1)両側仙腸開節炎　2-3度
• (2)片側仙腸関節炎　3-4度

0度:正常

1度:疑い

2度:軽度(小さな限局性の侵食像や硬化像)

3度:中等度(侵食像や硬化像の拡大,関節裂隙狭小)

4度:強直

• 2.HLA-B27陽性

• III. 除外診断

• 1. Reiter症候群
• 2. 乾癬性関節炎
• 3. 腸疾患合併関節炎
• 4. 反応性関節炎

• IV. 診断基準

• 確実例:主要症状1. 2, 3のうち1項目以上+必要検査1の(1)
• 疑い例:主要症状なし+必要検査1の(1)あるいは(2)

付記: HLA-B27の成績を記載のこと

治療

• 運動療法
• 薬物療法：NSAIDs。DMARDsも使われうる。

「ヒト白血球抗原」

　　[★]

英

human leucocyte antigen, HLA

同

ヒト白血球型抗原、HLA抗原 HLA antigen、ヒト組織適合性白血球抗原 human histocompatibility leukocyteantigen

主要組織適合抗原 MHC　←　免疫との関連はこちらを参照

関

骨髄バンク、HLA抗原

疾患との関連

出典不明

強直性脊椎炎	B27
関節リウマチ	DR4
重症筋無力症	DR9,DQ3
尋常性天疱瘡	A26,DR4
バセドウ病	DR5
I型糖尿病(インスリン依存性糖尿病)	B54,DR4,DR9,DR53,DQ4
グレーブス病	DR5
ベーチェット病	B51
原田病	DR4,DR53
潰瘍性大腸炎	B52,DR2
クローン病	DR4,DQ3
高安病	B52,DR2,DQ1
バージャー病	B52,DR2,DQ1
ナルコレプシー	DR2

first aid step1 2006 p.191

HLA-B27	psoriasis, ankylosing spondylitis, inflammatory bowel disease, Reiter's syndrome.
HLA-B8	Graves' disease, celiac sprue.
HLA-DR2	multiple sclerosis, hay fever, SLE, Goodpasture's syndrome.
HLA-DR3	diabetes mellitus type 1.
HLA-DR4	rheumatoid arthritis, diabetes mellitus type 1.
HLA-DR5	pernicious anemia → B12 deficiency, Hashimoto's thyroiditis.
HLA-DR7	steroid-responsive nephrotic syndrome.

まとめ

• HLA-DR4：自己免疫性肝炎 (YN.B-42)
• HLA-DR8：原発性胆汁性肝硬変 (YN.B-41)
• HLA-B27：強直性脊椎炎
• HLA-B51：ベーチェット病

• HLA-B39、HLA-B52：大動脈炎症候群

「rheumatoid spondylitis」

　　[★] リウマチ性脊椎炎

関

ankylosing spondylitis、Bechterew's disease

「Bechterew's disease」

　　[★]

関

ankylosing spondylitis

「Marie-Strumpell disease」

　　[★]

関

ankylosing spondylitis

「ankylosing」

　　[★]

• adj.

• 強直性の

関

ankylotic、tetanic、tonic