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.
医学词汇注释与简要讲解
vasculitis 血管炎
【suffix】'itis 炎症
appendicitis 阑尾炎
myocarditis 心肌炎
coronary aneurysms 冠状动脉瘤
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 心脏增大
heart failure 心功能不全,心衰
dipyridamole 双嘧达莫;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 猩红热
measles 麻疹
mononucleosis 单核细胞增多症
Stevens'Johnson syndrome
重症多形性红斑
erythema multiforme 多形性红斑
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
结节性多动脉炎
Takayasu’s arteritis 大动脉炎
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
假结核耶尔森菌
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,囊状的
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' 对抗的,反的
antipyretics 退热剂
antibiotic 抗生素
conjunctivitis 结膜炎
oropharyngeal erythema 口咽红斑
【prefix】de' 去除
desquamation脱屑;剥脱
deodorant 除臭剂
myocardial infarction 心肌梗死
gangrene 坏疽
renovascular 肾血管性
ileus 肠梗阻
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 冠脉搭桥
transplantation 移植
Prognosis
Death in <1%.
Chronic coronary insufficiency,premature atherosclerosis in <4%.
Favorable with early treatment with immunoglobulin,aspirin.