May-2015-CSBRP

Rheumatic Fever and

Rheumatic Heart Disease

CSBR.Prasad, MD.,

• 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

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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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)

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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Pathogenesis“Damage is mediated both by Abs and T-cells”

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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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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”

“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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“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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Rheumatic Heart Disease: Sreptococcal pharyngitis / tonsillitis

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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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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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

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

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Aschoff’s body

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Aschoff’s body

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Catterpillar chromatin in nuclei

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Catterpillar chromatin in nuclei

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Aschoff’s body – perivascular in location

Fibrinous pericarditis“Bread and butter” pericarditis

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Bread and butter

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Fibrinous pericarditis“Bread and butter” pericarditis

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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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Gross appearance of heart showing dilated left atrium with MacCallum plaque and vegetations

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Gross appearance of heart showing dilated left atrium with MacCallum plaque and vegetations

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Vegetations

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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

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]

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Mitral valve: MS [Button hole, Fish mouth]

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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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Acute and chronic rheumatic heart disease

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Mitral valve: MS [Button hole, Fish mouth]

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Rheumatic heart disease (shortening and thickening of chordae)

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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

Rheumatic fever: “Licks the joints and

Bites the heart”

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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

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• marantic endocarditis is a/w...• hypercoagulable states; involves

deposition of fibrin and platelets on leaflets of cardiac valves

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• 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)