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5.Rheumatic heart disease pathology
1. Rheumatic Heart DiseaseRheumatic Heart Disease
ďŹ 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.
ďŹ Of which heart is the most importantOf which heart is the most important
ďŹ 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.
ďŹ ââ RF licks the joints but bites the heart âRF licks the joints but bites the heart â
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2. Epidemiology Predisposing factorsEpidemiology Predisposing factors
ďŹ Ages 5-15 yrs are most susceptibleAges 5-15 yrs are most susceptible
ďŹ Rare <3 yrsRare <3 yrs
ďŹ Girls>boysGirls>boys
ďŹ Common in 3rd world countriesCommon in 3rd world countries
ďŹ Environmental factors-- over crowding, poor sanitation, poverty,Environmental factors-- over crowding, poor sanitation, poverty,
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3. Etiology & PathogenesisEtiology & Pathogenesis
ďŹ 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
ďŹ 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
ďŹ StreptococcalStreptococcal M proteinM protein react to cardiac myosinreact to cardiac myosin
ďŹ StreptoccocalStreptoccocal membrane antigenmembrane antigen cross react with myocardial & smoothcross react with myocardial & smooth
muscle sarcolemmamuscle sarcolemma
ďŹ 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
ďŹ Infection with group AInfection with group A
ββ hemolytic streptococci Sore throat(pharyngitis)hemolytic streptococci Sore throat(pharyngitis)
ďŹ Humoral immune response in 2-4 weeksHumoral immune response in 2-4 weeks
ďŹ 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. ďŹ 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 )
ďŹ Probably type III hypersensitivity reactionProbably type III hypersensitivity reaction
ďŹ Inflammatory lesion to myocardium, heart valves & other connectiveInflammatory lesion to myocardium, heart valves & other connective
tissuetissue
ďŹ Formation of aschoffâs bodyFormation of aschoffâs body
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7. Morphology /Aschoffâs Body(connective tissue cells)Morphology /Aschoffâs Body(connective tissue cells)
ďŹ Main characteristic feature in Rheumatic Fever is the formation ofMain characteristic feature in Rheumatic Fever is the formation of
Aschoffâs BodyAschoffâs Body
ďŹ Focal, inflammatory lesions are found in various sitesFocal, inflammatory lesions are found in various sites
Development of Aschoff's bodyDevelopment of Aschoff's body
ďŹ Exudative phase â there is focal fibrinoid necrosis & is seen onlyExudative phase â there is focal fibrinoid necrosis & is seen only
transiently in early phasetransiently in early phase
ďŹ Proliferative phase â leads to the formation of Aschoff's bodyProliferative phase â leads to the formation of Aschoff's body
ďŹ Healed phase â aschoffâs body are gradually replaced by fibrousHealed phase â aschoffâs body are gradually replaced by fibrous
scar in years time.scar in years time.
Aschoffâs bodies may be found in heart, Joints & subcutaneous tissueAschoffâs bodies may be found in heart, Joints & subcutaneous tissue
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8. Composition of Aschoffâs bodyComposition of Aschoffâs body
Central foci of fibrinoid necrosis surrounded byCentral foci of fibrinoid necrosis surrounded by
ďŹ Lymphocytes, Plasma cells, Aschoffâs cellsLymphocytes, Plasma cells, Aschoffâs cells
Aschoffâs cells/Anitschkow cells / cater-pillar cellsAschoffâs cells/Anitschkow cells / cater-pillar cells
ďŹ Activated histocytes(phagocyte cell) with abundant cytoplasm & centralActivated histocytes(phagocyte cell) with abundant cytoplasm & central
round-oval nucleiround-oval nuclei
ďŹ 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
Aschoffâs giant cellsAschoffâs giant cells
ďŹ Some of these are larger & multinucleated to form Aschoffâs giant cellsSome of these are larger & multinucleated to form Aschoffâs giant cells
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11. HeartHeart
ďŹ Carditis is the most important manifestations of rheumatic feverCarditis is the most important manifestations of rheumatic fever
ďŹ Aschofâs body may be found in all 3 layersAschofâs body may be found in all 3 layers
1. Pericarditis1. Pericarditis
ďŹ Diffuse fibrinous or serofibrinous pericartitis with bread & butterDiffuse fibrinous or serofibrinous pericartitis with bread & butter
appearanceappearance
ďŹ Aschcoffâs bodies are located in subserosal fat & fibrous tissueAschcoffâs bodies are located in subserosal fat & fibrous tissue
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12. ďŹ 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. ďŹ The typical âbread and butterâThe typical âbread and butterâ
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
ďŹ 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
ďŹ 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
ďŹ There may be dilatation of the ventricles, particularly mitral valve ringThere may be dilatation of the ventricles, particularly mitral valve ring
ďŹ 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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16. Joints / ArthritisJoints / Arthritis
ďŹ Large joints (typically the knees, ankles, elbows and wrists) of limbsLarge joints (typically the knees, ankles, elbows and wrists) of limbs
commonly affectedcommonly affected
ďŹ Sometimes spines, temporo-mandibularSometimes spines, temporo-mandibular
ďŹ Characterized by acute, painful, symmetric and migratory polyarthritisCharacterized by acute, painful, symmetric and migratory polyarthritis
ďŹ 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.
ďŹ Aschoffâs body may be found in synovia, capsule, tendon &Aschoffâs 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
ďŹ Erythema marginatumErythema marginatum occurs in less than 5% of patients.occurs in less than 5% of patients.
ďŹ 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.
ďŹ 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
ďŹ Subcutaneous nodulesSubcutaneous nodules occur in 5-7% of patients.occur in 5-7% of patients.
ďŹ 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)
ďŹ 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)
ďŹ 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.
ďŹ 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
ďŹ Diagnosis is based on presence of 2 or more major criteria orDiagnosis is based on presence of 2 or more major criteria or
ďŹ One major & 2 or more minor criteriaOne major & 2 or more minor criteria
ďŹ In both cases previous streptococcal infection is requiredIn both cases previous streptococcal infection is required
Major manifestationsMajor manifestations
ďŹ CarditisCarditis
ďŹ PolyarthritisPolyarthritis
ďŹ ChoreaChorea
ďŹ Erythema marginatumErythema marginatum
ďŹ 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 Ă-haemolytic streptococci (also fromThroat swab culture: group A Ă-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. ďŹ What are the effects of left & rt sided heart failureWhat are the effects of left & rt sided heart failure
ďŹ Pathogenesis of rheumatic feverPathogenesis of rheumatic fever
ďŹ 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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