出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/02/26 17:52:16」(JST)
閉塞性血栓性血管炎（へいそくせいけっせんせいけっかんえん、ラテン語Thromboangiitis Obliterans: TAO）は、末梢動脈に閉塞性の内膜炎を起こし、末梢部に潰瘍や壊疽を引き起こす病気。フェリックス・フォン・ヴィニヴァルターによって報告された。
一般に、発見者であるレオ・ビュルガーにちなんだ名前であるBuerger's diseaseとして、ビュルガー病（ドイツ語読み）或いはバージャー病（英語読み）で知られている。
日本においては、14番目の特定疾患治療研究対象疾患（難病）に定められており、国からの治療費等の補助が受けられる。
末梢動脈に血栓を生じ、それが結合組織に置き換えられて動脈が閉塞し血流の途絶を招き、結果的に末梢部の壊死を引き起こす。
東京医科歯科大学の研究調査により、病変部位より高率に歯周病菌スピロヘータ属 Treponema denticola が発見されていることが報告され、発症との因果関係が注目されている。
男性患者が9割であり、女性は少ない。20歳から50歳まで特に30代の喫煙者でストレスが過多な患者に多発。受動喫煙者を含めるとほぼ全員が喫煙に関与していると言う。日本には約1万人の罹患者がいるとされる。近年日本での患者は減少気味。
将来的に内膜炎が血管閉塞を引き起こし末梢を壊死させるのを防ぐ為に、血管の収縮を招くストレスに晒さない事が求められる。寒気を避け、温浴、マッサージ、運動等で血流を良くする事が求められる。また患部を清潔に保つ事も効果があるとされる。 喫煙は血管収縮を招く事から、絶対禁止が求められる。禁煙を遵守しない場合、足趾や下肢切断等に至る場合が多い。歯周病治療および口腔内ケアの徹底が望まれる。 医学的にもプロスタグランジンなど血小板凝縮抑制や血管拡張、血管バイパス形成等による血行再建手術、交感神経節切除などの対症療法が主流であるが、病変が末梢部にあるので血行再建手術は難しいとされる。現在自己造血幹細胞を用いた再生医療が試みられている。
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| この項目は、医学に関連した書きかけの項目です。この項目を加筆・訂正などしてくださる協力者を求めています（プロジェクト:医学／Portal:医学と医療）。 |
| Buerger's disease | |
|---|---|
| Classification and external resources | |
Complete occlusion of the right and stenosis of the left femoral artery as seen in a case of thromboangiitis obliterans
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| ICD-10 | I73.1 |
| ICD-9 | 443.1 |
| OMIM | 211480 |
| DiseasesDB | 1762 |
| MedlinePlus | 000172 |
| eMedicine | med/253 |
| MeSH | C14.907.137.870 |
Thromboangiitis obliterans (also known as Buerger's disease, Buerger disease [English /bʌrɡər/; German /byrgər/], or presenile gangrene[1] ) is a recurring progressive inflammation and thrombosis (clotting) of small and medium arteries and veins of the hands and feet. It is strongly associated with use of tobacco products,[2] primarily from smoking, but also from smokeless tobacco.
There is a recurrent acute and chronic inflammation and thrombosis of arteries and veins of the hands and feet. The main symptom is pain in the affected areas, at rest and while walking (claudication).[1] The impaired circulation increases sensitivity to cold. Peripheral pulses are diminished or absent. There are color changes in extremity. The colour may range from cyanotic blue to reddish blue. Skin becomes thin and shiny. Hair growth is reduced. Ulcerations and gangrene in the extremities are common complications, often resulting in the need for amputation of the involved extremity.[3]
There are characteristic pathologic findings of acute inflammation and thrombosis (clotting) of arteries and veins of the hands and feet (the lower limbs being more common). The mechanisms underlying Buerger's disease are still largely unknown, but smoking and tobacco consumption are major factors associated with it. It has been suggested that the tobacco may trigger an immune response in susceptible persons or it may unmask a clotting defect, either of which could incite an inflammatory reaction of the vessel wall.[4] This eventually leads to vasculitis and ischemic changes in distal parts of limbs.
A possible role for Rickettsia in this disease has been proposed.[5]
A concrete diagnosis of thromboangiitis obliterans is often difficult as it relies heavily on exclusion of other conditions. The commonly followed diagnostic criteria are outlined below although the criteria tend to differ slightly from author to author. Olin (2000) proposes the following criteria:[6]
Buerger’s disease can be mimicked by a wide variety of other diseases that cause diminished blood flow to the extremities. These other disorders must be ruled out with an aggressive evaluation, because their treatments differ substantially from that of Buerger’s disease. For Buerger’s there is no treatment known to be effective.
Diseases with which Buerger’s disease may be confused include atherosclerosis (build-up of cholesterol plaques in the arteries), endocarditis (an infection of the lining of the heart), other types of vasculitis, severe Raynaud's phenomenon associated with connective tissue disorders (e.g., lupus or scleroderma), clotting disorders of the blood, and others.
Angiograms of the upper and lower extremities can be helpful in making the diagnosis of Buerger’s disease. In the proper clinical setting, certain angiographic findings are diagnostic of Buerger’s. These findings include a “corkscrew” appearance of arteries that result from vascular damage, particularly the arteries in the region of the wrists and ankles. Collateral circulation gives "tree root" or "spider leg" appearance.[1] Angiograms may also show occlusions (blockages) or stenosis (narrowings) in multiple areas of both the arms and legs. Distal plethysmography also yields useful information about circulatory status in digits. To rule out other forms of vasculitis (by excluding involvement of vascular regions atypical for Buerger’s), it is sometimes necessary to perform angiograms of other body regions (e.g., a mesenteric angiogram).
Skin biopsies of affected extremities are rarely performed because of the frequent concern that a biopsy site near an area poorly perfused with blood will not heal well.
Smoking cessation has shown to slow the progression of the disease and decrease the severity of amputation in most patients,but does not halt the progression.
In acute cases, drugs and procedures which cause vasodilation are effective in reducing pain experienced by patient. For example, prostaglandins like Limaprost[8] are vasodilators and gives relief in pain, but do not help in changing the course of disease. Epidural anesthesia and hyperbaric oxygen therapy also have vasodilator effect.[1]
In chronic cases, Lumbar sympathectomy may be occasionally helpful.[9] It reduces vasoconstriction and increases blood flow to limb. It aids in healing and giving relief from pain of ischemic ulcers.[1] Bypass can sometimes be helpful in treating limbs with poor perfusion secondary to this disease. Use of vascular growth factor and stem cell injections have been showing promise in clinical studies. Debridement is done in necrotic ulcers. In gangrenous digits, amputation is frequently required. Above-knee and below-knee amputation is rarely required.[1]
Streptokinase has been proposed as adjuvant therapy in some cases.[10]
Despite the clear presence of inflammation in this disorder, anti-inflammatory agents such as corticosteroids have not been shown to be beneficial in healing, but do have significant anti-inflammatory and pain relief qualities in low dosage intermittent form. Similarly, strategies of anticoagulation have not proven effective.
Buerger's is not immediately fatal. Amputation is common and major amputations (of limbs rather than fingers/toes) are almost twice as common in patients who continue to smoke. Prognosis markedly improves if a person quits smoking. Female patients tend to show much higher longevity rates than men, as is in society. The only way to slow the progression of the disease is to abstain from all tobacco products.
The cause of the disease is thought to be autoimmune in nature and heavily linked to tobacco use in patients with Buerger's as primary disease. There have also been links to persons with digestive disorders.[citation needed]
Buerger's is more common among men than women. It is more common in Japan, India, and Manipur along the "old silk route" than in the United States and Europe.[citation needed] The disease is most common among South Asians.[citation needed] Incidence of thromboangiitis obliterans is 8 to 12 per 100,000 adults in the United States (0.75% of all patients with peripheral vascular disease).[citation needed]
Buerger's disease was first reported by Felix von Winiwarter in 1879 in Austria.[11] It wasn't until 1908, however, that the disease was given its first accurate pathological description, by Leo Buerger at Mount Sinai Hospital in New York City.[12] Buerger called it "presenile spontaneous gangrene" after studying amputations in 11 patients.
As reported by Alan Michie in God Save The Queen, published in 1952 (see pages 194 and following), King George VI was diagnosed with the disease in late 1948 and early 1949. Both legs were affected, the right more seriously than the left. The King's doctors prescribed complete rest and electric treatment to stimulate circulation, but as they were either unaware of the connection between the disease and smoking (the King was a heavy smoker) or unable to persuade the King to stop smoking, the disease failed to respond to their treatment. On March 12, 1949, the King underwent a lumbar sympathectomy, performed at Buckingham Palace by Dr. James R. Learmonth. The operation, as such, was successful, but the King was warned that it was a palliative, not a cure, and that there could be no assurance that the disease would not grow worse. From all accounts, the King continued to smoke.
The author and journalist John McBeth describes his experiences of the disease, and treatment for it, in a chapter called 'Year of the Leg' in his book entitled Reporter. Forty Years Covering Asia. [13]
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| リンク元 | 「閉塞性血栓性血管炎」 |
| 関連記事 | 「disease」 |
断を厳密に行う。
| ASO | TAO | |
| 好発年齢と性差 | 中高年の男性 (50歳以上) |
若年男性 (20-40歳) |
| 全身性合併症 (基礎疾患) |
高血圧,糖尿病,脂質異常症 | なし |
| 好発部位 | 大動脈分枝部～大腿動脈 (下肢の中枢側) |
膝窩動脈以下 (下肢の末梢側) |
| 遊走性静脈炎 | なし | あり |
| 喫煙 | 危険因子の一つ | 増悪 |
| 血管造影 | 虫食い像、動脈壁硬化 | 先細り像、Corkscrew状側副路。多発性分節的閉塞(閉塞は途絶状) |
| 石灰化 | 多い | 少ない |
| 予後 | 不良 | 良好 |