O slideshow foi denunciado.
Utilizamos seu perfil e dados de atividades no LinkedIn para personalizar e exibir anúncios mais relevantes. Altere suas preferências de anúncios quando desejar.

Acute Rheumatic Fever in children

1.262 visualizações

Publicada em

This presentation deals about Acute Rheumatic Fever in children.

Publicada em: Educação
  • Seja o primeiro a comentar

Acute Rheumatic Fever in children

  1. 1. ACUTE RHEUMATICACUTE RHEUMATIC FEVER in childrenFEVER in children
  2. 2. Plan of the lecture 1. Definition of acute rheumaticcute rheumatic feverfever 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
  3. 3. 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.
  4. 4. Chronic Rheumatic heart diseaseChronic Rheumatic heart disease (CRHD)(CRHD) -- 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
  5. 5. RISK FACTORS of ARFRISK FACTORS of ARF Age ofAge of 7-157-15 years oldyears old;; InheritanceInheritance;; PrematurityPrematurity;; 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)
  6. 6. ETIOLOGYETIOLOGY 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 typetype М1,М3,М5,М6,М14,М18,М19,М24,М27,М1,М3,М5,М6,М14,М18,М19,М24,М27, М29 (М29 (altogetheraltogether – 90– 90 types of M-types of M- proteinsproteins))..
  7. 7. β-β-hemolytic Streptococcus rolehemolytic Streptococcus role evidenceevidence  Constant connection of diseaseConstant connection of disease recurrence with recurrent exacerbationrecurrence with recurrent exacerbation of naso-pharyngeal Streptococcalof naso-pharyngeal Streptococcal infection.infection. 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..
  8. 8. PATHOGENESISPATHOGENESIS 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”)
  9. 9. 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.
  10. 10. Rheumatic process schemeRheumatic process scheme Exudative-alterative changesExudative-alterative changes (mucoid edema, fibrinoid(mucoid edema, fibrinoid necrosis)necrosis) Specific granulomaSpecific granuloma formation (perivascular information (perivascular in the myocardiumthe myocardium interstitium, endocardium,interstitium, endocardium, valves)valves) Evolution ofEvolution of granuloma duringgranuloma during 3-6 mo with3-6 mo with substitution bysubstitution by scar tissuescar tissue
  11. 11. ARF CLASSIFICATONARF CLASSIFICATON ((Russia association of rheumatologists,Russia association of rheumatologists, 2003 )2003 ) Clinic types Clinic signs Outcome Heart failure (HF), FC main additional 1.Acute rheumatic fever 2.Recurrent rheumatic fever Carditis Arthritis Chorea Erythema marginatum Rheumatic subcutaneous nodes Fever Arthralgia Abdominal syndrome Serosites Recovery CHD НF: 0, І, ІІА,ІІБ,ІІІ FC: 0, I, II, III, ІV,
  12. 12. Examples of diagnosis formationExamples of diagnosis formation  Acute rheumatic fever: carditis ( mitralAcute rheumatic fever: carditis ( mitral valvulitis). Migrated polyarthritis, HF1, (FC 1)valvulitis). Migrated polyarthritis, HF1, (FC 1)  Acute rheumatic feverAcute rheumatic fever :: choreachorea,, HF 0, (FC 0)HF 0, (FC 0)  Recurrent rheumatic feverRecurrent rheumatic fever:: carditis, combinedcarditis, combined mitral valve disease HF IImitral valve disease HF IIАА ((FCFC IIII).).  Chronic rheumatic heart diseaseChronic rheumatic heart disease:: combinedcombined mitral-aortic valve disease, HF IImitral-aortic valve disease, HF II Б (Б (FCFC IIIIII))
  13. 13. 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..
  14. 14. 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 affection)affection);; • 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 treatment).treatment).
  15. 15. RHEUMOCARDITISRHEUMOCARDITIS 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– endomyocarditis. Early symptoms of endomyocarditis  HR changes  Heart borders dilation (predominantly to left)  Apex beat decreasing  Gallop rhythm  Systolic murmur in 5 point and apex ( testify valvulitis)
  16. 16. 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
  17. 17. ECG SIGNS  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 interval)interval)  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 Phonocardiography:Phonocardiography: 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, mid-range)mid-range)
  18. 18. PERICARDITISPERICARDITIS  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::  FibrinogenousFibrinogenous  ExudativeExudative
  19. 19. Sydenham’s chorea criteriaSydenham’s chorea criteria:: Unvoluntary distal hyperkinesisUnvoluntary distal hyperkinesis Muscular hypotoniaMuscular hypotonia Impairment of coordinationImpairment of coordination Vegetative dysfunctionVegetative dysfunction Psychopathologic signsPsychopathologic signs
  20. 20. 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 checkedchecked
  21. 21. 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.
  22. 22. 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
  23. 23. Rheumatic process activityRheumatic process activity criteriacriteria High activityHigh activity ((ІІІІІІ gradegrade)) –– myocarditismyocarditis,, pericarditispericarditis,, polyarthritispolyarthritis,, pneumonia.pneumonia. Laboratory dataLaboratory data:: neutrophyl leucocytosisneutrophyl leucocytosis– 10-– 10- 12 · 10/912 · 10/9 gg//ll,, ESRESR –– more thanmore than 4040 mmmm//hh,, CRPCRP – 3-4– 3-4 plusespluses, ά-2-, ά-2-globulinsglobulins – 13-14%, γ-– 13-14%, γ- globulinsglobulins– 25%,– 25%, seromucoidsseromucoids – 0,2-0,6,– 0,2-0,6, DFADFA – 0,25 – 0,5– 0,25 – 0,5 IUIU,, ASLASL-О и-О и ASKASK –– higher ofhigher of normalnormal 3-53-5 timestimes..
  24. 24. Moderate activityModerate activity ((ІІІІ gradegrade)) –– moderatemoderate clinic signsclinic signs ((carditis symptoms, subfebrilecarditis symptoms, subfebrile temperature, polyarthritis ortemperature, polyarthritis or polyarthralgiapolyarthralgia).). 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 levellevel
  25. 25. DIAGNOSTIC CRITERIA of ARFDIAGNOSTIC CRITERIA of ARF Main criteriaMain criteria Minor criteriaMinor criteria Confirmative data forConfirmative data for ββHSAHSA CarditisCarditis PolyarthritisPolyarthritisитит ChoreaChorea Marginal erythemaMarginal erythema SubcutaneousSubcutaneous rheumatic nodulesrheumatic nodules ClinicClinic:: -- arthralgiaarthralgia;; -- feverfever.. Lab dataLab data:: --elevation of acuteelevation of acute phase indexesphase indexes:: ESR, CRPESR, CRP.. PR intervalPR interval elongation atelongation at ECGECG 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
  26. 26. 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)..
  27. 27. 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 weeks.weeks. 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.
  28. 28. Treatment stagesTreatment stages  1-1-stst–– hospitalizationhospitalization  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 disease.disease.
  29. 29. 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.
  30. 30. 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 rheumocarditis.rheumocarditis. 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.
  31. 31. 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  GlycosidesGlycosides  Chorea treatmentChorea treatment:: PhenobarbitalPhenobarbital,, group Bgroup B vitamins,vitamins, Natrii valproatisNatrii valproatis
  32. 32. Congestive heart disease therapyCongestive heart disease therapy  DiureticsDiuretics ((furesimidefuresimide),), thiazidethiazide ((hydrochlorthiazidehydrochlorthiazide),), potassium sparingpotassium sparing ((spironolactonespironolactone).).  Ca-channel blockersCa-channel blockers ((amlodipinamlodipin).).  Beta-adrenoblockersBeta-adrenoblockers ((metoprololmetoprolol))  GlycosidesGlycosides ((digoxindigoxin))
  33. 33. 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 tracttract..
  34. 34. 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 – RetarpenRetarpen)) 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.
  35. 35. 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
  36. 36. PrognosisPrognosis 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.

×