血管性水腫
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血管神經性水腫(英語:Angioedema)或血管性水腫是真皮、皮下組織、黏膜的局部腫脹。[1][3]可發生於面部、舌頭、喉、腹部、四肢。[1]常與蕁麻疹相關,蕁麻疹是皮膚的紅腫。[1][3]約數分鐘至數小時內發病。[1]
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血管神經性水腫 | |
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同義詞 | 血管性水腫,Angiooedema, Quincke's edema, angioneurotic edema |
血管神經性水腫: 患兒由於眼皮腫脹不能睜眼 | |
症狀 | 局部腫脹[1] |
起病年齡 | 幾分鐘到幾小時[1] |
類型 | 組胺調節, 緩激肽調節[1] |
風險因素 | 家族史[2] |
診斷方法 | 對症治療[2] |
鑑別診斷 | 全身型過敏性反應, 膿瘍, 接觸性皮炎[2] |
治療 | 插管, 環甲狀軟骨切開術(英語:cricothyroidotomy)[1] |
藥物 | 組胺: 抗組織胺藥, 皮質類固醇, 腎上腺素[1] 緩激肽: C1酯酶抑制物(英語:C1 esterase inhibitor), 艾卡拉肽(英語:ecallantide), 艾替班特(英語:icatibant), 新鮮冷凍血漿[1] |
盛行率 | ~100,000每年(美國)[1] |
分類和外部資源 | |
醫學專科 | 免疫學 |
ICD-9-CM | 995.1 |
OMIM | 106100、610618、106100、610618 |
DiseasesDB | 13606 |
MedlinePlus | 000846 |
eMedicine | 756261、135208、885100 |
[編輯此條目的維基數據] |
基本機制涉及組胺或緩激肽。[1]與組胺相關的是由於對過敏原的過敏反應,如蚊蟲叮咬、食物或藥品。[1]與緩激肽相關的是遺傳問題稱作獲得性C1酯酶抑制劑缺乏(英語:C1 esterase inhibitor deficiency),藥物有血管緊張素轉換酶抑制劑, 或淋巴組織增生性疾病(英語:lymphoproliferative disorder).[1]
為保護呼吸道通暢,對呼吸道特別是喉部發作水腫,必要時應進行氣管插管或環甲膜切開術。[1]組胺相關血管神經性水腫可抗組織胺藥:對症治療常採用抗組胺受體H1拮抗劑,對頑固的、應用抗組胺受體拮抗劑無效的患者,可合併應用抗組胺受體H2拮抗劑如西咪替丁(甲氰咪呱)或蘭替丁,有時可取得滿意效果。酮體芬亦可合併使用。擬交感神經藥物主要用於急性蕁麻疹和(或)神經性水腫,尤其是喉水腫患者,應用0.1%腎上腺素皮下注射,對嚴重急性過敏性反應可隔20~30分鐘注射。同時給予糖皮質類固醇激素靜脈滴注,氨茶鹼口服或靜脈注射。[1] 緩激肽相關的疾病可用C1酯酶抑制物(英語:C1 esterase inhibitor), 艾卡拉肽(英語:ecallantide), 艾替班特(英語:icatibant)治療。[1] 新鮮冷凍血漿也可作為替代療法。[1]美國每年約十萬人發生此病。[1]
症狀
![](http://upload.wikimedia.org/wikipedia/commons/thumb/7/7d/Blausen_0023_Angioedema.png/640px-Blausen_0023_Angioedema.png)
The skin of the face, normally around the mouth, and the mucosa of the mouth and/or throat, as well as the tongue, swell over the period of minutes to hours. The swelling can also occur elsewhere, typically in the hands. The swelling can be itchy or painful. There may also be slightly decreased sensation in the affected areas due to compression of the nerves. Urticaria (hives) may develop simultaneously.
In severe cases, stridor of the airway occurs, with gasping or wheezy inspiratory breath sounds and decreasing oxygen levels. Tracheal intubation is required in these situations to prevent respiratory arrest and risk of death.
Sometimes, the cause is recent exposure to an allergen (e.g. peanuts), but more often it is either idiopathic (unknown) or only weakly correlated to allergen exposure.
In hereditary angioedema, often no direct cause is identifiable, although mild trauma, including dental work and other stimuli, can cause attacks.[4] There is usually no associated itch or urticaria, as it is not an allergic response. Patients with HAE can also have recurrent episodes (often called "attacks") of abdominal pain, usually accompanied by intense vomiting, weakness, and in some cases, watery diarrhea, and an unraised, nonitchy splotchy/swirly rash. These stomach attacks can last one to five days on average, and can require hospitalization for aggressive pain management and hydration. Abdominal attacks have also been known to cause a significant increase in the patient's white blood cell count, usually in the vicinity of 13,000 to 30,000. As the symptoms begin to diminish, the white count slowly begins to decrease, returning to normal when the attack subsides. As the symptoms and diagnostic tests are almost indistinguishable from an acute abdomen (e.g. perforated appendicitis) it is possible for undiagnosed HAE patients to undergo laparotomy (operations on the abdomen) or laparoscopy (keyhole surgery) that turns out to have been unnecessary.
HAE may also cause swelling in a variety of other locations, most commonly the limbs, genitals, neck, throat and face. The pain associated with these swellings varies from mildly uncomfortable to agonizing pain, depending on its location and severity. Predicting where and when the next episode of edema will occur is impossible. Most patients have an average of one episode per month, but there are also patients who have weekly episodes or only one or two episodes per year. The triggers can vary and include infections, minor injuries, mechanical irritation, operations or stress. In most cases, edema develops over a period of 12–36 hours and then subsides within 2–5 days.
診斷
The diagnosis is made on the clinical picture. Routine blood tests (complete blood count, electrolytes, renal function, liver enzymes) are typically performed. Mast cell tryptase levels may be elevated if the attack was due to an acute allergic (anaphylactic) reaction. When the patient has been stabilized, particular investigations may clarify the exact cause; complement levels, especially depletion of complement factors 2 and 4, may indicate deficiency of C1-inhibitor. HAE type III is a diagnosis of exclusion consisting of observed angioedema along with normal C1 levels and function.
The hereditary form (HAE) often goes undetected for a long time, as its symptoms resemble those of more common disorders, such as allergy or intestinal colic. An important clue is the failure of hereditary angioedema to respond to antihistamines or steroids, a characteristic that distinguishes it from allergic reactions. It is particularly difficult to diagnose HAE in patients whose episodes are confined to the gastrointestinal tract. Besides a family history of the disease, only a laboratory analysis can provide final confirmation. In this analysis, it is usually a reduced complement factor C4, rather than the C1-INH deficiency itself, that is detected. The former is used during the reaction cascade in the complement system of immune defense, which is permanently overactive due to the lack of regulation by C1-INH.
Angioedema is classified as either hereditary or acquired.
獲得性血管性水腫
Acquired angioedema (AAE) can be immunologic, nonimmunologic, or idiopathic.[5] It is usually caused by allergy and occurs together with other allergic symptoms and urticaria. It can also occur as a side effect to certain medications, particularly ACE inhibitors. It is characterized by repetitive episodes of swelling, frequently of the face, lips, tongue, limbs, and genitals. Edema of the gastrointestinal mucosa typically leads to severe abdominal pain; in the upper respiratory tract, it can be life-threatening.[6]
遺傳型血管性水腫
Hereditary angioedema (HAE) exists in three forms, all of which are caused by a genetic mutation inherited in an autosomal dominant form. They are distinguished by the underlying genetic abnormality. Types I and II are caused by mutations in the SERPING1 gene, which result in either diminished levels of the C1-inhibitor protein (type I HAE) or dysfunctional forms of the same protein (type II HAE). Type III HAE has been linked with mutations in the F12 gene, which encodes the coagulation protein factor XII. All forms of HAE lead to abnormal activation of the complement system, and all forms can cause swelling elsewhere in the body, such as the digestive tract. If HAE involves the larynx, it can cause life-threatening asphyxiation.[7] The pathogenesis of this disorder is suspected to be related to unopposed activation of the contact pathway by the initial generation of kallikrein and/or clotting factor XII by damaged endothelial cells. The end product of this cascade, bradykinin, is produced in large amounts and is believed to be the predominant mediator leading to increased vascular permeability and vasodilation that induces typical angioedema "attacks".[8]