cvs rhd-csbrp
TRANSCRIPT
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May-2015-CSBRP
Rheumatic Fever and
Rheumatic Heart Disease
CSBR.Prasad, MD.,
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• Arthritis• Arthralgia • Types of Streptococci• What is beta hemolysis?• Markers for Streptococcal infection• What are the diseases caused by Streptococci ?• When do you clinically suspect pericarditis / pleurisy?• How to differentiate these two?
May-2015-CSBRP
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Diseases caused by Streptococcus
• Pneumonia• Necrotizing fasciitis• Rheumatic fever• Poststreptococcal glomerulonephritis• Pharyngitis / tonsillitis• Neonatal meningitis (Group-B)• PANDAS / Tourette syndrome : Pediatric
Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections
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StreptococcusTypes of Hemolysis
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May-2015-CSBRP
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May-2015-CSBRP
Rheumatic fever (RF)• It is an acute, immunologically mediated
disease • Occur a few weeks after group A
Streptococcal pharyngitis• Multisystemic disorder• May progress to chronic RHD (Valvular
heart disease)
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May-2015-CSBRP
Rheumatic fever (RF)
• It is an acute, immunologically mediated disease
• Occur a few weeks after group A Streptococcal pharyngitis
• Streptococcus strains: 1,3,5,6 & 18 [Griffith type]
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May-2015-CSBRP
Pathogenesis“Damage is mediated both by Abs and T-cells”
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May-2015-CSBRP
MORPHOLOGY
Acute RF• Aschoff bodies• Pancraditis • Verrucous
vegetations• MacCallum plaques
Chronic RHD• Valvular changes
– Leaflet thickening, – Commissural fusion
and shortening, and – Thickening and fusion
of the tendinous cords
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RHD
Valves affected are: decreasing order– Mitral– Aortic– Tricuspid– Pulmonary
RHD is virtually the only cause of mitral stenosis
Mnemonic: MAT
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May-2015-CSBRP
Clinical Features
RF is characterized by:– Migratory polyarthritis of the large joints– Pancarditis– Subcutaneous nodules– Erythema marginatum– Sydenham’s chorea
The diagnosis of RF is established by the “Jones criteria”
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“Jones criteriaJones criteria”Required CriteriaEvidence of antecedent Strep infection: ASO / Strep antibodies / Strep group A throat culture / Recent scarlet fever / anti-deoxyribonuclease B / anti-hyaluronidaseMajor Diagnostic Criteria
CarditisPolyarthritisChoreaErythema marginatumSubcutaneous Nodules
Minor Diagnostic CriteriaFeverArthralgiaPrevious rheumatic fever or rheumatic heart diseaseAcute phase reactions: ESR / CRP / LeukocytosisProlonged PR interval
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“Jones criteriaJones criteria”Diagnostic : 1 Required Criteria and 2 Major Criteria and 0 Minor Criteria
Diagnostic :1 Required Criteria and 1 Major Criteria and 2 Minor Criteria
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May-2015-CSBRP
“Jones criteriaJones criteria”
• Evidence of a preceding group A streptococcal infection
+• Two of the major manifestations or
• One major and two minor manifestations
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May-2015-CSBRP
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May-2015-CSBRP
Rheumatic Heart Disease: Sreptococcal pharyngitis / tonsillitis
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Erythema marginatum
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Subcutaneous nodules
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Sydenham's chorea: causes loss of muscle control, leading to awkward gait and distorted hand gestures
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May-2015-CSBRP
Acute RF
• Appears 10 days to 6 weeks after a group A Streptococcal infection
• Children between ages 5 -15yrs• Pharyngeal cultures for streptococci are
negative • Indirect evidence of Streptococcal infection:
– ASLO– DNase B
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May-2015-CSBRP
Acute RF • The predominant clinical manifestations are:
– Carditis and– Arthritis
• Arthritis:– More common in adults than in children– Migratory polyarthritis
• “Acute carditis”: – Pericardial friction rubs– Tachycardia, and – Arrhythmias
• Myocarditis:– Cardiac dilation with functional MR or – Heart failure
• Approximately 1% of affected individuals die of fulminant RF involvement of the heart
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RHD - Microscopy
• Characteristic feature of RHD is Aschoff’s body
• Aschoff’s body composed of:– Swollen eosinophilic collagen– T-cells– Plasma cells– Plump macrophages – Anitschkow cells– They are perivascular in location
May-2015-CSBRP
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May-2015-CSBRP
Aschoff’s body
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May-2015-CSBRP
Aschoff’s body
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May-2015-CSBRP
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May-2015-CSBRP
Catterpillar chromatin in nuclei
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May-2015-CSBRP
Catterpillar chromatin in nuclei
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May-2015-CSBRP
Aschoff’s body – perivascular in location
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Fibrinous pericarditis“Bread and butter” pericarditis
May-2015-CSBRP
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Bread and butter
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Fibrinous pericarditis“Bread and butter” pericarditis
May-2015-CSBRP
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RHD - Microscopy
• Fibrinoid necrosis: seen in the endocardium, cusps, along the tendinous cords
• Vegetations: Small projections on the lines of closure
• MacCollum’s patches: Irregular thickening in the left atrial wall in the presence of MR
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May-2015-CSBRP
Gross appearance of heart showing dilated left atrium with MacCallum plaque and vegetations
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May-2015-CSBRP
Gross appearance of heart showing dilated left atrium with MacCallum plaque and vegetations
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May-2015-CSBRP
Vegetations
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May-2015-CSBRP
Acute RF
• After an initial attack there is increased vulnerability to reactivation of the disease with subsequent pharyngeal infections
• Damage to the valves is cumulative• Clinical manifestations appear years or
even decades after the initial episode of RF
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Chronic RHD
• Characterized by organization of acute inflammation and subsequent fibrosis
• Valves show thickening, commissural fusion and shortening,
• Cordae tendinae shows thickening and shortening
• Mitral valve: MS [Button hole, Fish mouth]
May-2015-CSBRP
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May-2015-CSBRP
Mitral valve: MS [Button hole, Fish mouth]
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May-2015-CSBRP
Rheumatic mitral stenosis
• “Fish mouth” or “Button hole” stenoses• Left atrial enlargement• Mural thrombi in left atrium• Long standing MS: pulmonary vascular
and parenchymal changes > RVH• Valves:
– Organization of the acute inflammation– Neovascularization and – Transmural fibrosis
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May-2015-CSBRP
Acute and chronic rheumatic heart disease
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May-2015-CSBRP
Mitral valve: MS [Button hole, Fish mouth]
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May-2015-CSBRP
Rheumatic heart disease (shortening and thickening of chordae)
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May-2015-CSBRP
Complications
• Cardiac murmurs• Cardiac hypertrophy and dilation• Valvular heart disease• Heart failure• Arrhythmias (particularly AF in the setting
of mitral stenosis)• Thromboembolic complications• Infective endocarditis
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Rupture of chordae tendinae
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Mural Thrombus in the left atrium
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Rheumatic fever: “Licks the joints and
Bites the heart”
May-2015-CSBRP
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E N D
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Sydenham’s Chorea• Extrapyramidal disorder: • Fast, clonic involuntary movements (especially face and
limbs)• Muscular hypotonus• Emotional lability• First sign: difficulty walking, talking, writing• Usually a late manifestation, can be months after infection• May be the only manifestation of ARF• Often associated with carditis• Usually benign and resolves in 2-3 months• But can last for more than 2 years
May-2015-CSBRP
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May-2015-CSBRP
• marantic endocarditis is a/w...• hypercoagulable states; involves
deposition of fibrin and platelets on leaflets of cardiac valves
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May-2015-CSBRP
• this endocarditis has vegetations on both sides of the valve surface
• libman-sacks endocarditis (a/w lupus)
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• these vegetations have fibrinoid necrosis and inflammation and are located on both sides of the valve surface
• libman-sacks endocarditis (lupus)