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Transcript
Rheumatism
Infective endocarditis
Chronic valvular vitium of the heart
Pathology Department
Zhejiang University School of Medicine
Zhu keqing 竺可青
[email protected]
2013-3-18
RHEUMATISM
An immunologically mediated disease,
related to an episode of group A (β-hemolytic)
streptococcal pharyngitis.
Multisystem inflammatory disease, heart and joints
are favored sites.
Rheumatic fever, acute rheumatic carditis
Repetitive attacks lead to chronic valvular deformities
The pathologic feature is Aschoff bodies.
The pathogenetic sequence and key morphologic features
of acute rheumatic heart disease
ESSENTIAL MORPHOLOGY
(1) alteration and exudation
Serous, fibrinous exudate, and
leukocytes infiltration, mucoid
degeneration and fibrinoid necrosis.
Outcomes:
Complete resolution
Fibrosis
Granulomas
(2) Proliferation or granulomas
Hallmark: Aschoff bodies
Fibrinoid necrosis, Anitschkow cells
Aschoff giant cells
Lymphocytes
Plasma cells
Aschoff body
• During acute RF, focal inflammatory lesions are found in various
tissues. They are most distinctive within the heart, where they are
called Aschoff bodies.
• They consist of foci of swollen eosinophilic collagen surrounded by
lymphocytes (primarily T cells), occasional plasma cells, and plump
macrophages called Anitschkow cells (pathognomonic for RF).
• These distinctive cells have abundant cytoplasm and central roundto-ovoid nuclei in which the chromatin is disposed in a central,
slender, wavy ribbon (hence the designation “caterpillar cells”).形态
• Some of the larger macrophages become multinucleated to form
Aschoff giant cells. 巨细胞
Aschoff body
Aschoff bodies
(3) Fibrosis
Aschoff bodies spindle scar
Rheumatic endocarditis
Sites: mitral and aortic valves
Fibrinoid necrosis
Vegetations: small, warty, along the
line of closure
Precipitation of fibrin
Organization, fibrosis
Thickening, shortening,
commissural fusion of
leaflets and tendinous
cord
Rheumatic myocarditis
Perivascular
Aschoff body
Rheumatic
pericarditis
Serous exudate
pericardial effusion
Fibrinous exudate
Cor villosum
Constrictive pericarditis
Rheumatic arthritis
Serous inflammation
Complete resolution
Large joints: migratory
Local signs: arthralgia
Self-limited, no chronic deformity
Rheumatic arteritis
Often involves small arteries,
Fibrinoid necrosis
Mononuclear infiltration
Aschoff bodies
Fibrosis
Lumen narrowing
CNS changes
Rheumatic arteritis,
Subcortical encephalitis
Minor chorea / Sydenham chorea
(a neurologic disorder with involuntary
purposeless, rapid movements)
ACUTE:
-Inflammation
-Aschoff bodies
-Anitschkow cells
-Pancarditis
-Vegetations on
chordae tendinae at
leaflet junction
CHRONIC:
THICKENED VALVES
COMMISURAL FUSION
THICK, SHORT,
CHORDAE TENDINAE
CLINICAL FEATURES
•
•
•
•
•
Migratory Polyarthritis
Myocarditis
Subcutaneous nodules
Erythema marginatum
Sydenham chorea
风心小结
(肉牙肿性炎)3 stages
• 变质渗出期---黏液样变/纤维素样坏死---1月
• 增生期/肉牙肿期---风湿细胞/Aschoff cell/ Aschoff body---2/3月
• 纤维化期/愈合期---4/6月
Rheumatic heart disease (小结)
1 Rheumatic endocarditis
---valvulitis---vegetations白色血栓
---verrucous endocarditis疣状心内膜炎
---McCallum斑
2 Rheumatic myocarditis ---Aschoff body
3 Rheumatic pericarditis---绒毛心Cor villosum ---constrictive
pericarditis
4 Rheumatic pancarditis
•
•
•
•
•
•
Rheumatic arthritis –游/大/无-舔关节咬心脏
Erythema annullare 环形红斑-非特异性渗出性炎
Subcutaneous nodules 皮下结节-风湿小体
Rheumatic arteritis-风湿小体
Chorea minor 小舞蹈症
皮质下脑炎
感染性心内膜炎 Infective endocarditis
病原微生物直接侵袭心内膜特别
是心瓣膜而引起的心内膜炎
急性感染性心内膜炎
亚急性感染性心内膜炎
急性感染性心内膜炎
病原:致病力强的化脓菌
金黄色葡萄球菌、溶血性链球菌、肺炎球菌
心瓣膜:发生在原来无病变的正常心内膜
主要累及二尖瓣、主动脉瓣
临床上:起病急,发展快,病程短,死亡率高
亚急性感染性心内膜炎
病原:致病力较小病原微生物
草绿色链球菌,肠球菌,真菌等。
心瓣膜:发生在原来有病变的心内膜
主要累及二尖瓣、主动脉瓣
菜花状或息肉状疣状赘生物:
纤维素、血小板、NC、坏死物
细菌团
血管:动脉栓塞和血管炎;
肾: 微栓塞致灶性肾小球肾炎。
临床上:病程长,数月或1年
ABE/SBE区别
• Acute endocarditis describes a destructive, tumultuous infection,
frequently of a previously normal heart valve, with a highly virulent
organism, that leads to death within days to weeks of more than
50% of patients despite antibiotics and surgery.
• In contrast, organisms of low virulence can cause infection in a
previously abnormal heart, particularly on deformed valves. In such
cases, the disease may appear insidiously and, even untreated,
pursue a protracted course of weeks to months (subacute
endocarditis). Most patients with subacute IE recover after
appropriate antibiotic therapy.
Morphology.
• In both the subacute and acute forms of the disease, friable, bulky,
and potentially destructive vegetations containing fibrin,
inflammatory cells, and bacteria or other organisms are present on
the heart valves.
• Osler小结-皮下小动脉炎
• Systemic emboli may occur at any time because of the friable
nature of the vegetations, and they may cause infarcts in the brain,
kidneys, myocardium, and other tissues.
• Because the embolic fragments contain large numbers of virulent
organisms, abscesses often develop at the sites of such infarcts
(septic infarcts).
Chronic valvular vitium of the heart
心瓣膜病
• Vavular stenosis
• Valvular insufficiency
• Stenosis is the failure of a valve to open
completely, thereby impeding forward flow.
• Insufficiency, in contrast, results from
failure of a valve to close completely,
thereby allowing reversed flow.
• Aortic stenosis: calcification of anatomically
normal and congenitally bicuspid aortic valves.
• Aortic insufficiency: dilation of the ascending
aorta, related to hypertension and aging.
• Mitral stenosis: rheumatic heart disease.
• Mitral insufficiency: myxomatous degeneration
(mitral valve prolapse).
70% of all VHD
• AS
– Calcification of a deformed valve
– “Senile” calcific AS
– Rheum, Heart Dis.
• MS
–Rheumatic Heart Disease
二尖瓣狭窄X线检查
左心房增大 胸骨
左缘第3肋间心浊音
界增大,使心腰消
失,如梨形,称二
尖瓣型心(三大一
小—梨型心)
风心病二狭,瓣口面积1.2cm2
主动脉瓣关闭不全X线检查
左心室增大 向左下增大,
心腰加深,似靴形:见于
主动脉瓣关闭不全
男,36岁,风心病,主动脉瓣关闭不全
左心室80mm
AORTIC STENOSIS
2X gradient pressure
LVH (but no hypertension), ischemia
Cardiac decompensation, angina, CHF
50% die in 5 years if angina present
50% die in 2 years if CHF present
MITRAL ANNULAR
CALCIFICATION
• Calcification of the
mitral “skeleton”
• Usually NO
dysfunction
• Regurgitation
usually, but
Stenosis possible
• F>>M
REGURGITATIONS
• AR
– Rheumatic
– Infectious
– Aortic dilatations
• Syphilis
• Rheumatoid Arthritis
• Marfan
• MR
–MVP
–
–
–
–
Infectious
Fen-Phen
Papillary muscles, chordae tendinae
Calcification of mitral ring (annulus)
Mitral Valve Prolapse (MVP)
• MYXOMATOUS degeneration of the
mitral valve
• Associated with connective tissue
disorders
• “Floppy” valve
• 3% incidence, F>>M
• Easily seen on echocardiogram
MVP: CLINICAL FEATURES
•
•
•
•
•
•
Usually asymptomatic
Mid-systolic “click”
Holosystolic murmur if regurg. present
Occasional chest pain, dyspnea
97% NO untoward effects
3% Infective endocarditis, mitral
insufficiency, arrythmias, sudden death
VEGETATIONS
• INFECTIVE >5mm
• NON-Infective <5mm
ARTIFICIAL VALVES
• Mechanical
• Xenografts (porcine)
• 60% have complications within 10
years
人工瓣膜置换术后并发症
•
•
•
•
•
•
1. PVE
2. Thrombosis and thromboembolism
3. Hemorrhage
4. Perivalvular leakage
5. AVB
6. Prosthetic valve functional disturbance