Key facts

Synonym:mucocutaneous lymph node syndrome.

Definition:acute systemic vasculitis syndrome affecting small and medium'sized arteries (coronaries).

Classic imaging appearance:coronary aneurysms.

More common in Japan,Asian populations.

醫(yī)學(xué)詞匯注釋與簡要講解

vasculitis 血管炎

【suffix】'itis 炎癥

appendicitis 闌尾炎

myocarditis 心肌炎

coronary aneurysms 冠狀動脈瘤

Imaging findings

Best imaging clue:multiple fusiform and saccular coronary aneurysms.

Chest radiography

Initially normal.

Cardiomegaly and signs of congestive heart failure with development of myocarditis or myocardial ischemia.

Echocardiography

Aneurysmal dilatation of proximal coronary arteries in 20%.

Myocardial dysfunction with ischemia.

CT finding:multislice helical CT angiography can depict coronary aneurysms.

MR findings

Cardiac'gated T1WI:myocardial wall tinning,recently infarcted myocardium enhances with gadolinium.

Gradient'echo (GRE) cine MRI shows regional wall motion abnormality.

Gadolinium'MRA can depict coronary aneurysms.

Thallium myocardial perfusion imaging (SPECT)：pharmacological stress testing with dipyridamole can demonstrate myocardial ischemia.

Coronary angiography

Multiple fusiform and saccular coronary aneurysms.

Regression of the aneurysms or development of fixed stenoses.

Acute thrombotic occlusion of a coronary may be treated percutaneously.

cardiomegaly 心臟增大

heart failure 心功能不全，心衰

dipyridamole 雙嘧達(dá)莫；潘生丁

Imaging recommendations

Frequent echocardiographic screening of the proximal coronary arteries.

Coronary angiography with progressive disease and development of ischemia.

Differential diagnosis

Other exanthematous infections

Septic shock syndrome,scarlet fever,measles,mononucleosis.

Allergies,hypersensitivity reactions

Drugs,Stevens'Johnson syndrome,erythema multiforme.

scarlet fever 猩紅熱

measles 麻疹

mononucleosis 單核細(xì)胞增多癥

Stevens'Johnson syndrome

重癥多形性紅斑

erythema multiforme 多形性紅斑

Fig.1 a)VR image shows left and right coronary aneurysms (arrows).b)Axial CT images shows dilation of the left main coronary artery and focal thickening and peripherally calcification on arterial wall (arrow).c)Curved planar reconstruction of right coronary artery shows the multiple dilation of the lumen and extensive thickening and peripherally calcification on the wall (arrows).Notice the filling defect due to the thrombus.Fig.2 Chest X'ray film shows calcifications (arrows) represent calcified coronary artery aneurysms.

Other vasculitides

Systemic lupus erythematosus(SLE),polyarteritis nodosa,Takayasu’s arteritis.

polyarteritis nodosa

結(jié)節(jié)性多動脈炎

Takayasu’s arteritis 大動脈炎

Pathology

General path comments

Most common cause of acquired coronary artery disease in children.

Self'limiting vasculitis with multi'organ involvement.

Etiology'pathogenesis

Immune response to unknown infectious agent.

Associated with Yersinia pseudotuberculosis,Epstein'Barr virus.

Pathogenesis similar to scarlet fever and toxic shock syndrome.

Abnormal antigenic response to unknown bacterial toxin,virus.

Acquired T'cell abnormalities.

Epidemiology

Japan:incidence 80/100,000 children less than 4 year.

Also prevalent in other countries,affecting mainly Asian populations.

Suggestion of minor epidemic outbursts every 3'4 years.

Minimal seasonal variations (more in winter and spring).

self'limiting 自限性

Yersinia pseudotuberculosis

假結(jié)核耶爾森菌

Epstein'Barr virus EB 病毒

Gross pathologic'surgical features

Saccular dilatation of origins of coronary arteries,beaded appearance.

Microscopic features

Perivasculitis involving small vessels.

Larger vessels become secondarily inflamed,with aneurysm and thrombus formation.

Chronic thrombosis,scar formation.

saccular 囊的，囊狀的

Clinical Issues

Presentation

Duration of illness approximately 7 weeks.

High fever more than 5 days,refractory to antipyretics.

Within 3 days:conjunctivitis,painful cervical lymphadenopathy,oropharyngeal erythema,strawberry tongue.

After 3 days:characteristic rash with desquamation of hands and feet.

Acute cardiac involvement:myocarditis (36%),pericardial effusion (16%).

Coronary artery involvement:(1)transient coronary dilatation (25%);(2) coronary aneurysms(16%),regression in half;(3)myocardial infarction(1%'2%),leading to death in 0.5%.

Other systemic artery involvement (2%).

Brachial:gangrene of fingers.

Renal:renovascular hypertension.

Iliac,mesenteric:ileus.

Also cholecystitis,cholangitis,pancreatitis,sialadenitis.

【prefix】anti' 對抗的，反的

antipyretics 退熱劑

antibiotic 抗生素

conjunctivitis 結(jié)膜炎

oropharyngeal erythema 口咽紅斑

【prefix】de' 去除

desquamation脫屑;剝脫

deodorant 除臭劑

myocardial infarction 心肌梗死

gangrene 壞疽

renovascular 腎血管性

ileus 腸梗阻

sialadenitis 涎腺炎

Natural history

Self'limiting disease in majority of cases.

May progress to chronic myocardial ischemia,infarction.

Treatment

Intravenous immunoglobulin,aspirin.

Transcatheter coronary intervention (thrombolysis,balloon angioplasty).

Occasionally:coronary bypass surgery or cardiac transplantation.

immunoglobulin 免疫球蛋白

aspirin 阿司匹林

coronary bypass 冠脈搭橋

transplantation 移植

Prognosis

Death in <1%.

Chronic coronary insufficiency,premature atherosclerosis in <4%.

Favorable with early treatment with immunoglobulin,aspirin.