Rheumatic heart disease pathology

1. Rheumatic Heart DiseaseRheumatic Heart Disease
 Rheumatic fever is an acute post streptococcal immune mediatedRheumatic fever is an acute post streptococcal immune mediated
inflammatory disease of connective tissues.inflammatory disease of connective tissues.
 Of which heart is the most importantOf which heart is the most important
 Permanent heart damage from repeated attacks of rheumatic feverPermanent heart damage from repeated attacks of rheumatic fever
is called rheumatic heart disease.is called rheumatic heart disease.
 ““ RF licks the joints but bites the heart “RF licks the joints but bites the heart “
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2. Epidemiology Predisposing factorsEpidemiology Predisposing factors
 Ages 5-15 yrs are most susceptibleAges 5-15 yrs are most susceptible
 Rare <3 yrsRare <3 yrs
 Girls>boysGirls>boys
 Common in 3rd world countriesCommon in 3rd world countries
 Environmental factors-- over crowding, poor sanitation, poverty,Environmental factors-- over crowding, poor sanitation, poverty,
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3. Etiology & PathogenesisEtiology & Pathogenesis
 Acute Rheumatic fever follows 2-4 weeks after Group AAcute Rheumatic fever follows 2-4 weeks after Group A ββ-hemolytic-hemolytic
streptococci throat infectionstreptococci throat infection
 Is the result of immune response to antigens of rheumatogenic strainsIs the result of immune response to antigens of rheumatogenic strains
of Streptococcusof Streptococcus
The following antigens of streptoccocci cross react to connective tissueThe following antigens of streptoccocci cross react to connective tissue
antigensantigens
 StreptococcalStreptococcal M proteinM protein react to cardiac myosinreact to cardiac myosin
 StreptoccocalStreptoccocal membrane antigenmembrane antigen cross react with myocardial & smoothcross react with myocardial & smooth
muscle sarcolemmamuscle sarcolemma
 Hyaluronate capsuleHyaluronate capsule of streptococcus cross react with heart valveof streptococcus cross react with heart valve
glycoproteinglycoprotein
Probably there occurs type III immune complex reactionProbably there occurs type III immune complex reaction
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4. Steps or sequence in PathogenesisSteps or sequence in Pathogenesis
 Infection with group AInfection with group A
ββ hemolytic streptococci Sore throat(pharyngitis)hemolytic streptococci Sore throat(pharyngitis)
 Humoral immune response in 2-4 weeksHumoral immune response in 2-4 weeks
 Production of antistreptococcal antibodies against the antigen group AProduction of antistreptococcal antibodies against the antigen group A ββ
hemolytic streptococcihemolytic streptococci
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5.  A cross reaction takes place between the streptococcal antibody &A cross reaction takes place between the streptococcal antibody &
connective tissue antigen (Due to similarity of streptococcal antigen withconnective tissue antigen (Due to similarity of streptococcal antigen with
that of cardiac tissue or other connective tissue )that of cardiac tissue or other connective tissue )
 Probably type III hypersensitivity reactionProbably type III hypersensitivity reaction
 Inflammatory lesion to myocardium, heart valves & other connectiveInflammatory lesion to myocardium, heart valves & other connective
tissuetissue
 Formation of aschoff’s bodyFormation of aschoff’s body
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6. 66

7. Morphology /Aschoff’s Body(connective tissue cells)Morphology /Aschoff’s Body(connective tissue cells)
 Main characteristic feature in Rheumatic Fever is the formation ofMain characteristic feature in Rheumatic Fever is the formation of
Aschoff’s BodyAschoff’s Body
 Focal, inflammatory lesions are found in various sitesFocal, inflammatory lesions are found in various sites
Development of Aschoff's bodyDevelopment of Aschoff's body
 Exudative phase – there is focal fibrinoid necrosis & is seen onlyExudative phase – there is focal fibrinoid necrosis & is seen only
transiently in early phasetransiently in early phase
 Proliferative phase – leads to the formation of Aschoff's bodyProliferative phase – leads to the formation of Aschoff's body
 Healed phase – aschoff’s body are gradually replaced by fibrousHealed phase – aschoff’s body are gradually replaced by fibrous
scar in years time.scar in years time.
Aschoff’s bodies may be found in heart, Joints & subcutaneous tissueAschoff’s bodies may be found in heart, Joints & subcutaneous tissue
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8. Composition of Aschoff’s bodyComposition of Aschoff’s body
Central foci of fibrinoid necrosis surrounded byCentral foci of fibrinoid necrosis surrounded by
 Lymphocytes, Plasma cells, Aschoff’s cellsLymphocytes, Plasma cells, Aschoff’s cells
Aschoff’s cells/Anitschkow cells / cater-pillar cellsAschoff’s cells/Anitschkow cells / cater-pillar cells
 Activated histocytes(phagocyte cell) with abundant cytoplasm & centralActivated histocytes(phagocyte cell) with abundant cytoplasm & central
round-oval nucleiround-oval nuclei
 Chromatin in center of the nucleus appears as a slender wavy ribbon &Chromatin in center of the nucleus appears as a slender wavy ribbon &
resembles a caterpillarresembles a caterpillar
Aschoff’s giant cellsAschoff’s giant cells
 Some of these are larger & multinucleated to form Aschoff’s giant cellsSome of these are larger & multinucleated to form Aschoff’s giant cells
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11. HeartHeart
 Carditis is the most important manifestations of rheumatic feverCarditis is the most important manifestations of rheumatic fever
 Aschof’s body may be found in all 3 layersAschof’s body may be found in all 3 layers
1. Pericarditis1. Pericarditis
 Diffuse fibrinous or serofibrinous pericartitis with bread & butterDiffuse fibrinous or serofibrinous pericartitis with bread & butter
appearanceappearance
 Aschcoff’s bodies are located in subserosal fat & fibrous tissueAschcoff’s bodies are located in subserosal fat & fibrous tissue
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12.  Fibrinous pericarditis: Gross, natural color, an excellent example ofFibrinous pericarditis: Gross, natural color, an excellent example of
bread and butter appearancebread and butter appearance
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13.  The typical “bread and butter”The typical “bread and butter”
appearance of fibrinousappearance of fibrinous
pericarditispericarditis
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This is an example of a fibrinous
pericarditis. The surface appears
roughened from the normal glistening
appearance by the strands of pink-tan
fibrin.

14. 2. Myocarditis2. Myocarditis
 Aschoff's bodies are scattered(diffusion) within interstitial connectiveAschoff's bodies are scattered(diffusion) within interstitial connective
tissue often in relation to intra myocardial blood vesselstissue often in relation to intra myocardial blood vessels
 Adjacent myocytes(long tubular cell that develop form muscles) mayAdjacent myocytes(long tubular cell that develop form muscles) may
be damaged or killedbe damaged or killed
 There may be dilatation of the ventricles, particularly mitral valve ringThere may be dilatation of the ventricles, particularly mitral valve ring
 Death due to cardiac failure may occur in the acute stage due toDeath due to cardiac failure may occur in the acute stage due to
myocarditismyocarditis
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15. 3. Endocarditis3. Endocarditis
Next classNext class
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16. Joints / ArthritisJoints / Arthritis
 Large joints (typically the knees, ankles, elbows and wrists) of limbsLarge joints (typically the knees, ankles, elbows and wrists) of limbs
commonly affectedcommonly affected
 Sometimes spines, temporo-mandibularSometimes spines, temporo-mandibular
 Characterized by acute, painful, symmetric and migratory polyarthritisCharacterized by acute, painful, symmetric and migratory polyarthritis
 The joints are involved in quick succession for a day and up to 4 weeks.The joints are involved in quick succession for a day and up to 4 weeks.
 Aschoff’s body may be found in synovia, capsule, tendon &Aschoff’s body may be found in synovia, capsule, tendon &
fascia(sheet of connective tissue fibers,primarily collagen,that formsfascia(sheet of connective tissue fibers,primarily collagen,that forms
beneath the skin to attach,stabilize,enclose,separate muscles and otherbeneath the skin to attach,stabilize,enclose,separate muscles and other
internal organs)internal organs)
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17. Skin lesionsSkin lesions
 Erythema marginatumErythema marginatum occurs in less than 5% of patients.occurs in less than 5% of patients.
 The lesions start as red macules(scar) which fade in the centre butThe lesions start as red macules(scar) which fade in the centre but
remain red at the edges and occur mainly on the trunk and proximalremain red at the edges and occur mainly on the trunk and proximal
extremities but not the face.extremities but not the face.
 The resulting red rings or 'margins' may coalesce or overlap.The resulting red rings or 'margins' may coalesce or overlap.
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18. Skin lesionsSkin lesions
 Subcutaneous nodulesSubcutaneous nodules occur in 5-7% of patients.occur in 5-7% of patients.
 They are small (0.5-2.0 cm), firm and painless, and are best feltThey are small (0.5-2.0 cm), firm and painless, and are best felt
over extensor surfaces of bone or tendons. (elbow, wrist, ankle)over extensor surfaces of bone or tendons. (elbow, wrist, ankle)
 Nodules typically appear more than 3 weeks after the onset of otherNodules typically appear more than 3 weeks after the onset of other
manifestationsmanifestations
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19. Sydenham's choreaSydenham's chorea (St Vitus dance)(St Vitus dance)
 Characterized by rapid, irregular, aimless, involuntary movements ofCharacterized by rapid, irregular, aimless, involuntary movements of
the muscles of the limbs, face, and trunk.the muscles of the limbs, face, and trunk.
 This is a late neurological manifestationThis is a late neurological manifestation
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20. Clinical featuresClinical features
JONES CRITERIA FOR THE DIAGNOSIS OF RHEUMATIC FEVERJONES CRITERIA FOR THE DIAGNOSIS OF RHEUMATIC FEVER
 Diagnosis is based on presence of 2 or more major criteria orDiagnosis is based on presence of 2 or more major criteria or
 One major & 2 or more minor criteriaOne major & 2 or more minor criteria
 In both cases previous streptococcal infection is requiredIn both cases previous streptococcal infection is required
Major manifestationsMajor manifestations
 CarditisCarditis
 PolyarthritisPolyarthritis
 ChoreaChorea
 Erythema marginatumErythema marginatum
 Subcutaneous nodulesSubcutaneous nodules
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21. Minor manifestationsMinor manifestations
• FeverFever
• Arthralgia(pain in joints)Arthralgia(pain in joints)
• Previous rheumatic feverPrevious rheumatic fever
• Raised ESR or CRP(erythrocyte sedimrntation rate in which b/d cellRaised ESR or CRP(erythrocyte sedimrntation rate in which b/d cell
sediment in a 1hr.)/(C-reactive protein is a marker of inflammation insediment in a 1hr.)/(C-reactive protein is a marker of inflammation in
the body,therefore its level in the inc. if there is any inflammation inthe body,therefore its level in the inc. if there is any inflammation in
the body.)the body.)
• LeucocytosisLeucocytosis
• First-degree AV(ateioventricular) block seen in ECG.First-degree AV(ateioventricular) block seen in ECG.
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22. INVESTIGATIONS IN ACUTE RHEUMATIC FEVERINVESTIGATIONS IN ACUTE RHEUMATIC FEVER
Evidence of a systemic illness (non-specific)Evidence of a systemic illness (non-specific)
• Leucocytosis, raised ESR, raised CRPLeucocytosis, raised ESR, raised CRP
Evidence of preceding streptococcal infection (specific)Evidence of preceding streptococcal infection (specific)
• Throat swab culture: group A ß-haemolytic streptococci (also fromThroat swab culture: group A ß-haemolytic streptococci (also from
family members and contacts)family members and contacts)
• Antistreptolysin o(is the antibody made against streptolysin,anAntistreptolysin o(is the antibody made against streptolysin,an
immunogenic,oxygen-labile hemolytic toxin produced by mostimmunogenic,oxygen-labile hemolytic toxin produced by most
strains of group A and many strrains of groups C &G streptococci)strains of group A and many strrains of groups C &G streptococci)
antibodies (ASO titres): rising titres, or levels of > 200 U (adults) orantibodies (ASO titres): rising titres, or levels of > 200 U (adults) or
> 300 U (children)> 300 U (children)
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23.  What are the effects of left & rt sided heart failureWhat are the effects of left & rt sided heart failure
 Pathogenesis of rheumatic feverPathogenesis of rheumatic fever
 Write down the morphology of Rheumatic fever / write short notes onWrite down the morphology of Rheumatic fever / write short notes on
rheumatic feverrheumatic fever
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