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ACUTE RHEUMATICACUTE RHEUMATIC
FEVER in childrenFEVER in children
Plan of the lecture
1. Definition of acute rheumaticcute rheumatic
2. Risk factors and e2. Risk factors and etiologytiology
3. Pathogenesis3. Pathogenesis
4. Classification4. Classification
5. Diagnostic criteria5. Diagnostic criteria
6. Treatment and prophylaxis6. Treatment and prophylaxis
Acute rheumatic fever (ARF)Acute rheumatic fever (ARF)
– is postinfectious complication of tonsillitis
(angina) or pharyngitis induced by β-β-
hemolytic Streptococcus group A (hemolytic Streptococcus group A (ββHSA) inHSA) in
the form of systemic inflammation ofthe form of systemic inflammation of
connective tissue. It develops in predisposedconnective tissue. It develops in predisposed
patients of 7-15 years old due to autoimmunepatients of 7-15 years old due to autoimmune
response toresponse to ββHSA antigens and crossHSA antigens and cross
reactivity to the similar autoantigens ofreactivity to the similar autoantigens of
affected human tissues.affected human tissues.
Chronic Rheumatic heart diseaseChronic Rheumatic heart disease
-- is the disease of affectedis the disease of affected
cardiac valves presented by post-cardiac valves presented by post-
inflammatory edge fibrosis ofinflammatory edge fibrosis of
valves or heart valvesvalves or heart valves
insufficiency and/or stenosis thatinsufficiency and/or stenosis that
has been formed after acutehas been formed after acute
rheumatic feverrheumatic fever
RISK FACTORS of ARFRISK FACTORS of ARF
Age ofAge of 7-157-15 years oldyears old;;
Inherited connective tissue anomalies,Inherited connective tissue anomalies,
abnormality of collagen fibersabnormality of collagen fibers;;
Unfavorable surroundings (living in wetUnfavorable surroundings (living in wet
houses with low temperature)houses with low temperature)
ARF is developed after angina orARF is developed after angina or
pharyngitis, caused by rheumatic strainspharyngitis, caused by rheumatic strains
––ββHSA with high contagiousness.HSA with high contagiousness.
Most “rheumatic” strains has specificMost “rheumatic” strains has specific
M-proteins in their membrane of theM-proteins in their membrane of the
М29 (М29 (altogetheraltogether – 90– 90 types of M-types of M-
β-β-hemolytic Streptococcus rolehemolytic Streptococcus role
Constant connection of diseaseConstant connection of disease
recurrence with recurrent exacerbationrecurrence with recurrent exacerbation
of naso-pharyngeal Streptococcalof naso-pharyngeal Streptococcal
Stable increasing of ASL-O, ASH, ASKStable increasing of ASL-O, ASH, ASK
in patient’s serumin patient’s serum
High preventive efficacy of adequateHigh preventive efficacy of adequate
antibiotic therapy of A-Streptococcalantibiotic therapy of A-Streptococcal
tonsillitis, pharyngitistonsillitis, pharyngitis..
ARF progression is defined by:ARF progression is defined by:
1.1. Direct toxic damage of myocardium byDirect toxic damage of myocardium by
“cardiotropic”“cardiotropic” ββHSA enzymes.HSA enzymes.
2.2. Immune response forImmune response for ββHSA antigensHSA antigens
and synthesis of anti-streptococcusand synthesis of anti-streptococcus
antibodies with cross reaction to antigensantibodies with cross reaction to antigens
of human tissues (“phenomenon ofof human tissues (“phenomenon of
molecular mimicry”)molecular mimicry”)
Antigen mimicry formationAntigen mimicry formation
Specific products (toxins) of Streptococcus –Specific products (toxins) of Streptococcus –
Streptolysin, Hyaluronidase, Erythrogene toxin hasStreptolysin, Hyaluronidase, Erythrogene toxin has
cytotoxic and immune reactive properties and inducecytotoxic and immune reactive properties and induce
damage of tissues, production of antitoxic antibodies,damage of tissues, production of antitoxic antibodies,
supression of phagocytosissupression of phagocytosis
Circulated antibodies affect microvasculature withCirculated antibodies affect microvasculature with
destructive-productive vasculitisdestructive-productive vasculitis
Cross reactive antibodies are formed and they canCross reactive antibodies are formed and they can
affect as well Streptococcus and tissues.affect as well Streptococcus and tissues.
Autoimmune process is induced with affection ofAutoimmune process is induced with affection of
connective tissue.connective tissue.
Rheumatic process schemeRheumatic process scheme
Exudative-alterative changesExudative-alterative changes
(mucoid edema, fibrinoid(mucoid edema, fibrinoid
Specific granulomaSpecific granuloma
formation (perivascular information (perivascular in
the myocardiumthe myocardium
interstitium, endocardium,interstitium, endocardium,
Evolution ofEvolution of
granuloma duringgranuloma during
3-6 mo with3-6 mo with
substitution bysubstitution by
scar tissuescar tissue
ARF CLASSIFICATONARF CLASSIFICATON
((Russia association of rheumatologists,Russia association of rheumatologists, 2003 )2003 )
Clinic types Clinic signs Outcome Heart
НF: 0, І,
FC: 0, I, II,
RHEUMATIC POLYARTHRITISRHEUMATIC POLYARTHRITIS
Is a main syndrome inIs a main syndrome in 2/32/3 of all cases in ARF.of all cases in ARF.
Usually knee and ankle or wrist and ulna jointsUsually knee and ankle or wrist and ulna joints
are affectedare affected
Pains are very severe that can cause siniviitisPains are very severe that can cause siniviitis
and periarticular tissues affectionand periarticular tissues affection
InIn 10-15%10-15% of all cases there is polyartritisof all cases there is polyartritis
Rheumatic polyarthritis is characterised byRheumatic polyarthritis is characterised by
benign course with migration of inflammatorybenign course with migration of inflammatory
affection, more frequently symmetric likeaffection, more frequently symmetric like
oligoarthritis or more rare like monoarthritis.oligoarthritis or more rare like monoarthritis...
Rheumatic arthritis usually is combined withRheumatic arthritis usually is combined with
rheumocarditis or chorearheumocarditis or chorea..
Rheumatic polyarthritis peculiaritiesRheumatic polyarthritis peculiarities
• Big and middle size joints involving into pathologicBig and middle size joints involving into pathologic
process ( more frequently knees, ankles, wrists or ulnaprocess ( more frequently knees, ankles, wrists or ulna
• Dissociation of vague clinic signs and acute subjectiveDissociation of vague clinic signs and acute subjective
symptoms – prominent pains in affected joints especiallysymptoms – prominent pains in affected joints especially
during movementsduring movements;;
• Symmetric affectionSymmetric affection;;
• Migration of painsMigration of pains;;
• AbsenceAbsence ооf deformity formationf deformity formation;;
• Prompt restitution after anti-inflammatory treatmentPrompt restitution after anti-inflammatory treatment
(pains can disappear several hours after anti-rheumatic(pains can disappear several hours after anti-rheumatic
is the main ARF syndrome that defines severity
and outcome of disease.
It can be affected all the layers of heart, but the
main carditis manifestation is affection of
myocardium and endocardium–
Early symptoms of endomyocarditis
Heart borders dilation (predominantly to left)
Apex beat decreasing
Systolic murmur in 5 point and apex ( testify
Systolic murmur of mitral regurgitation is theSystolic murmur of mitral regurgitation is the
main rheumatic valvulitis symptommain rheumatic valvulitis symptom
It’s characterized as prolonged and blowingIt’s characterized as prolonged and blowing;;
Different in intensity and not dependant onDifferent in intensity and not dependant on
body position and respiration phasebody position and respiration phase;;
Connected with 1 soundConnected with 1 sound;;
Covers the main part of systoleCovers the main part of systole;;
Best auscultation is at apexBest auscultation is at apex;;
Can be conducted to left axillary regionCan be conducted to left axillary region
Rhythm disorders- tachy- bradycardia, prematureRhythm disorders- tachy- bradycardia, premature
beats, sinus node migrationbeats, sinus node migration
Impairment of AV conductivity ( elongation ofImpairment of AV conductivity ( elongation of РР-R-R
Changes of waveChanges of wave ТТ : it can be moderately decreased or: it can be moderately decreased or
pick-like in left leadspick-like in left leads
Voltage decrease ofVoltage decrease of QRSQRS testify about process severitytestify about process severity
1 sound amplitude decreasing in apex1 sound amplitude decreasing in apex
Increased amplitude of 3 and 4 soundIncreased amplitude of 3 and 4 sound
Functional systolic murmur ( connected with 1 sound,Functional systolic murmur ( connected with 1 sound,
Usually it’s combined withUsually it’s combined with
endomyocarditis and testifies ofendomyocarditis and testifies of
polyserositis affectionpolyserositis affection
Types of rheumatic pericarditisTypes of rheumatic pericarditis::
Filatov testFilatov test –– “eyes and tongue” symptom“eyes and tongue” symptom»»
Cherni symptomCherni symptom –– belly retraction duringbelly retraction during
inspiration instead of its protrusioninspiration instead of its protrusion
Johihess testJohihess test –– patient and doctor extendpatient and doctor extend
hands each to other and child repeathands each to other and child repeat
movements after doctormovements after doctor
““Flabby shoulders” symptom,Flabby shoulders” symptom,
“jackknife”symptom, “folding hand”“jackknife”symptom, “folding hand”
symptom- are the symptoms to revealsymptom- are the symptoms to reveal
muscular hypotonicitymuscular hypotonicity
To reveal coordination abnormality –To reveal coordination abnormality –
finger-nose, knee-calcaneal, Rombergfinger-nose, knee-calcaneal, Romberg
teststests,, rotation abnormality must berotation abnormality must be
Skin affectionSkin affection
Erythema marginatum –light pinkish rashErythema marginatum –light pinkish rash
presented with thin contour more bright externalpresented with thin contour more bright external
margin and light inner one, not elevated on skin ,margin and light inner one, not elevated on skin ,
don’t disappear after pressing to it. Rash isn’tdon’t disappear after pressing to it. Rash isn’t
stable, painless.stable, painless.
Rheumatic nodules – round, dense, painlessRheumatic nodules – round, dense, painless
structures with sizes that ranges from several mmstructures with sizes that ranges from several mm
to 1-2 cm, localized in subcutaneous layer, fscii,to 1-2 cm, localized in subcutaneous layer, fscii,
aponeurosis , in periostal zones or bursa,aponeurosis , in periostal zones or bursa,
predominantly on extensor surfaces.predominantly on extensor surfaces.
ARF Recurrent attack clinicsARF Recurrent attack clinics
Episode of Streptococcal infection precedesEpisode of Streptococcal infection precedes
recurrent ARF attackrecurrent ARF attack
Onset is acute in the case of chorea appearanceOnset is acute in the case of chorea appearance
subacute onsetsubacute onset
Every next attack usually repeat previous oneEvery next attack usually repeat previous one
in clinics and activityin clinics and activity
Signs of carditis worsen comparatively toSigns of carditis worsen comparatively to
previous attack with increasing of murmursprevious attack with increasing of murmurs
and appearing of new valve affection signsand appearing of new valve affection signs
Cardiac failure progressesCardiac failure progresses
Moderate activityModerate activity ((ІІІІ gradegrade)) –– moderatemoderate
clinic signsclinic signs ((carditis symptoms, subfebrilecarditis symptoms, subfebrile
temperature, polyarthritis ortemperature, polyarthritis or
Laboratory data:Laboratory data: neutrophyl leucocytosisneutrophyl leucocytosis– 10– 10
· 10/9· 10/9 gg//ll,, ESRESR ––20-30 mm20-30 mm//hh,, CRPCRP ––1-21-2
plusespluses, ά-2-, ά-2-globulinsglobulins –– 11-11-13%, γ-13%, γ-
globulinsglobulins––22-22-25%,25%, seromucoidsseromucoids – 0,2-0,6,– 0,2-0,6,
DFADFA – 0,25 – 0,– 0,25 – 0,33 IUIU,, ASLASL-О и-О и ASKASK ––
higher of normal 1,5-2higher of normal 1,5-2 timestimes..
ММinimal activityinimal activity ((ІІ gradegrade)) –– without strikingwithout striking
clinic signs, lab data are increased but notclinic signs, lab data are increased but not
significantly or can be of higher normalsignificantly or can be of higher normal
DIAGNOSTIC CRITERIA of ARFDIAGNOSTIC CRITERIA of ARF
Main criteriaMain criteria Minor criteriaMinor criteria
Confirmative data forConfirmative data for ββHSAHSA
Marginal erythemaMarginal erythema
rheumatic nodulesrheumatic nodules
Lab dataLab data::
--elevation of acuteelevation of acute
phase indexesphase indexes::
ESR, CRPESR, CRP..
PR intervalPR interval
elongation atelongation at
Positive A-STR. Strain bacterialPositive A-STR. Strain bacterial
test from oral cavity and/ortest from oral cavity and/or
positive fast test of A-Str.positive fast test of A-Str.
antigenantigen.. ElevatedElevated ββHSAHSA
antibodies titers.antibodies titers.
(Kissel Jones criteria and review of RRA* 2003)
** Russian Rheumatologist AssociationRussian Rheumatologist Association
Differentiative diagnosisDifferentiative diagnosis
If you can’t find quite clear connection withIf you can’t find quite clear connection with
preceding Streptoccocus type A infectionpreceding Streptoccocus type A infection
differentiate disease with viral myocarditisdifferentiate disease with viral myocarditis
(Cocsakie B)(Cocsakie B)
Mitral valve prolapse (especially ifMitral valve prolapse (especially if
hypermobility of joints is present)hypermobility of joints is present)
Infectious endocirditis and cardiac mixoma.Infectious endocirditis and cardiac mixoma.
Reactive arthritis induced by intestine or urineReactive arthritis induced by intestine or urine
genital infection.genital infection.
Lime- disease: arthritis, carditis, CNS and skinLime- disease: arthritis, carditis, CNS and skin
affection ( it is caused by Borrelia burgdorferi)affection ( it is caused by Borrelia burgdorferi)..
Treatment common approachTreatment common approach
Hospitalization, bed regimen for 3 weeks. InHospitalization, bed regimen for 3 weeks. In
severe carditis activity is permitted aftersevere carditis activity is permitted after
congestive heart disease signs will disappear andcongestive heart disease signs will disappear and
ESR will decrease to 25 mm/h, CRP (-) for 2ESR will decrease to 25 mm/h, CRP (-) for 2
Diet is restricted in salt and decreasedDiet is restricted in salt and decreased
carbohydrates, enriched by proteins ( not lesscarbohydrates, enriched by proteins ( not less
than 1 g/kg), vitamins and K+.than 1 g/kg), vitamins and K+.
ARF is integrated and consist of etiologic,ARF is integrated and consist of etiologic,
pathogenic and symptomatic therapy altogetherpathogenic and symptomatic therapy altogether
with restitution therapy.with restitution therapy.
Treatment stagesTreatment stages
2-2-ndnd –– local sanatorium ( sanitation of chroniclocal sanatorium ( sanitation of chronic
infection focuses, physical training metabolicinfection focuses, physical training metabolic
therapy, secondary prophylaxis) to gettherapy, secondary prophylaxis) to get
complete remission.complete remission.
3-3-dd –– dispensary observation in polyclinics todispensary observation in polyclinics to
prevent recurrent attacks and progression ofprevent recurrent attacks and progression of
ETIOLOGIC TREATMENTETIOLOGIC TREATMENT
Directed forDirected for ββHSA eradication.HSA eradication.
Penicyllines are the best choice.Penicyllines are the best choice.
ChildrenChildren:: BenzylpenicyllineBenzylpenicylline,, 400-600400-600 000000
IU/day IM QID for 10 days.IU/day IM QID for 10 days.
AdolescentsAdolescents:: BenzylpenicyllineBenzylpenicylline, 1,5-4, 1,5-4
million IU/day QID/TID for 10 days.million IU/day QID/TID for 10 days.
If medication intolerance is present:If medication intolerance is present:
macrolides or oral cefalosporins.macrolides or oral cefalosporins.
Pathogenic therapyPathogenic therapy
Main goalsMain goals::
--To suppress activity of rheumatic process.To suppress activity of rheumatic process.
--Prevention of chronic heart disease on the ground ofPrevention of chronic heart disease on the ground of
NSAIDS – acetylsalycylic acid 1-1,5 g/day not more thanNSAIDS – acetylsalycylic acid 1-1,5 g/day not more than
2 g/day TID for 1,5 -2 mo.2 g/day TID for 1,5 -2 mo.
-Prednisone-Prednisone 0,7-0,80,7-0,8 mg/kg/day once per day aftermg/kg/day once per day after
breakfast for 2 weeks, later dosage steadily is decreasedbreakfast for 2 weeks, later dosage steadily is decreased
by 2,5 mg every 5-7 days.by 2,5 mg every 5-7 days.
After GCS treatment is over diclofenac orally 2-3After GCS treatment is over diclofenac orally 2-3
mg/kg/day TID for 1,5-2 mo long.mg/kg/day TID for 1,5-2 mo long.
If chronic heart disease risk is present in lingering courseIf chronic heart disease risk is present in lingering course
aminochinolones can be prescribed.aminochinolones can be prescribed.
Symptomatic treatmentSymptomatic treatment
Potassium and magnesia aspartatisPotassium and magnesia aspartatis 3-63-6 tabtab../day/day
Tid forTid for 11momo.. InozinInozin 0,6-1,20,6-1,2 g/day forg/day for 11momo..
NandrolonNandrolon 11mlml intramuscularintramuscular once per weekonce per week,,
1010 injection for courseinjection for course.. CocarboxylaseCocarboxylase,, Co-Co-
enzymeenzyme,, lipoic acidlipoic acid
Chorea treatmentChorea treatment:: PhenobarbitalPhenobarbital,, group Bgroup B
vitamins,vitamins, Natrii valproatisNatrii valproatis
Primary prophylaxisPrimary prophylaxis
The main prophylaxis consist ofThe main prophylaxis consist of
antimicrobial treatment ofantimicrobial treatment of acute andacute and
chronic recurrentchronic recurrent ββHSA of respiratoryHSA of respiratory
Secondary prophylaxisSecondary prophylaxis
Prevention of recurrent attacks and disease progression inPrevention of recurrent attacks and disease progression in
patients with ARF and provided by regular intake ofpatients with ARF and provided by regular intake of
long-acting Penicyllines (Benzatyl Benzylpenicylline –long-acting Penicyllines (Benzatyl Benzylpenicylline –
For children of body weight less than 25 kg -For children of body weight less than 25 kg -600600000 IU/IM000 IU/IM
once peronce per 33 weeksweeks..
For children with body weight more than 25 kg -For children with body weight more than 25 kg -1,21,2mlnmln
IU I/M once per 3 weeksIU I/M once per 3 weeks..
AdolescentsAdolescents - 2,4- 2,4 mln IU I/M once per 3 weeksmln IU I/M once per 3 weeks..
Alternative scheme – Erythromycine 40 mg/kg/day orally.Alternative scheme – Erythromycine 40 mg/kg/day orally.
Duration of secondary prophylaxisDuration of secondary prophylaxis
For patients with ARF without carditis (arthritis,For patients with ARF without carditis (arthritis,
chorea)chorea) - 5- 5 years after last attack or within 18 yearsyears after last attack or within 18 years
old ( choose that is longer)old ( choose that is longer)
In case of recovered carditis without chronic heartIn case of recovered carditis without chronic heart
disease – for 10 years after last attack or within 25disease – for 10 years after last attack or within 25
years old ( choose the longer term)years old ( choose the longer term)
For patients with chronic heart diusease ( even afterFor patients with chronic heart diusease ( even after
surgery) – lifelongsurgery) – lifelong
Prophylaxis is madeProphylaxis is made year-round not seasonallyyear-round not seasonally
Direct life threatening due to ARF is absentDirect life threatening due to ARF is absent
( except of rare cases of pancarditis in( except of rare cases of pancarditis in
children). Mostly prognosis is defined by heartchildren). Mostly prognosis is defined by heart
condition ( degree and severity of chronic heartcondition ( degree and severity of chronic heart
disease, valves lesion, congestive heart failure).disease, valves lesion, congestive heart failure).
Of great significance are the term of treatmentOf great significance are the term of treatment
onset. If therapy is delayed or absentonset. If therapy is delayed or absent
probability of chronic heart disease is higher.probability of chronic heart disease is higher.