Rheumatic Fever

Rheumatic FeverDr. Sadewantoro. Sp.JPCardiologist Medical Faculty, Hang Tuah University.

Demam Rheuma Akut (DRA)Suatu keradangan difus sebagai komplikasi lambat dari radang tenggorokan non supuratif o.k. streptokokus grup APenyakit Jantung Reumatik Suatu penyakit jantung akibat dari Demam Rheuma Akut yang meninggalkan gejala sisa berupa kerusakan katub jantungManifestasi MayorKarditisPoliartritisChoreaEritema marginatumNodul subkutanDx berdasarkan Kriteria Jones 2 Mayor / 2 Minor + 1 MayorManifestasi MinorDemam Rheuma sebelumnyaArtralgiaFebrisLab: LED, leukositosisEKG: interval PR memanjangGejala sisa pada katub tidak terjadi bila DRA ditangani awal dengan penatalaksanaan yang benar.Kelainan Katub Jantung akibat DRA sering mengenai:Katub Mitral 75-80%Katub Aorta 30%Katub Tricuspid 5%Katub Pulmonal

Kelainan bisa berupa stenosis atau regurgitasi (insufisiensi) yang akan memberi suara bisingRHEUMATIC FEVERCause Rheumatic Heart Disease (RHD)

Systemic disease, non supuratif Febris attack with intermitten remision, at Beta Haemolitikus group A,RHEUMATIC FEVERGroup of disease :Colagen Hypersensitif

Joint Heart ImportantSkin Serebral

RHEUMATIC FEVERINSIDENS

Asll ages90 % 5-15 yearsRare < 4 th.Male female

Low Sosio EconomicHigh populationClaimedGizi

EPIDEMIOLOGYInfection of the skin - younger than 6 yrStreptococcal pharyngitis - between 5 and 15 yr of ageScarlet fever - common in children > 3 yr of ageRHEUMATIC FEVER DEFINITIONAutoimmune disease occurring as consequence of infection with group A beta hemolytic streptococcus

Mainly affects children ages 6- 15

Capsule Cell wallProtein antigens Group carbohydratePeptidoglycanCyto.membraneCytoplasm Antigen of outer protein cell wall of GABHS induces antibody response in victim which result in autoimmune damage to heart valves, sub cutaneous tissue,tendons, joints & basal ganglia of brainETIOLOGYAETHIOPATHOGENESIS

C. Anti-M antibodies against the streptococci may cross-react with heart tissue, causing the pancarditis that is observed in RF.B. Rheumatogenic strains of GABHS M types l, 3, 5, 6,18 & 24 have antigenic domains similar to antigens in components of the human heartA. Only infections GABHS of the pharynx initiate or reactivate RF.PathophysiologyOccurs 2- 3 weeks after infection with group A beta hemotytic strep (strep throat).

The strep organism stimulates an autoimmune response in which autoantibodies attack:MyocardiumPericardiumMitral valveJointsCentral nervous system

HistologicallyFocal collections of inflammatory cells (Aschoff bodies) thru'out heart,esp. LA. These comprise macrophages, plasma cells & some lymphocytes.

Valve leaflets are thickened & fused.

Clinical Features:Acute Rheumatic Fever Acute Inflammatory PhaseHeart Pancarditis (40-50%)Skin Erythema Marginatum/ S.nodule (10%)CNS Sydenham Chorea (15%)Migratory polyarthritis (75%)Chronic Rheumatic Fever Deforming fibrotic valvular disease.

Signs and SymptomsGeneral symptomsfatigue, malaise, fever,anorexiaSpecific symptoms (depends on organ system affected)

Carditissystolic murmur, abnormal EKG, CHFPolyarthritisjoint symptoms and subcutaneous nodules over jointsChoreaabnormal involuntary movements caused byCNS damage (St. Vitus Dance)Erythema marginatumpink, macular rashSubcutan noduleSymptoms (acute RF)Heart symptoms (60%): Sleeping tachycardia changing murmurs pericardial rub heart failure cardiomegaly conduction defects (45-70%) apical systolic murmur Carey Coomb's murmur (mid-diastolic) due to thickening of mitral valve leaflets Esp. causes stenosis in mitral (70%), aortic (40%), tricuspid (10%) and pulmonary (2%). Myocarditis ArrhythmiasOther symptoms:Migratory (flitting) large joint polyarthritis = red and v. tender joints (75%)

Subcutaneous nodules (2-20%)

Erythema marginatum = trunk, thighs and arms = 'bathing suit distrib.' (2-10%)

Sydenham's chorea = odd darting movements in late RF, often preceded by emotional lability & uncharacteristic behaviour. Commoner in women and aka St Vitus' dance.Infeksi akibat hemolitikus Streptokokus Grup ARheumatic Fever

CarditisPeriode silent (2mgg)

Infeksi Tenggorokan

Infeksi Kuman Hemolitikus StreptokokusGroup A

PolyarthritisChoreaErythema marginatumSub Kutan ModulRheumatic Heart DiseaseElectrocardiogram Persistent sinus tachycardiaSinus bradycardiaProlonged PR intervalTransient complete heart blockAtrial fibrillation or flutterBundle branch blockLow QRS voltage

DiagnosisEvidence of recent strep. infection:Recent scarlet feverPositive growth from throat swabIncrease in antistreptolysin O titre (ASOT) >200u/ml.DiagnosisMajor criteria:

Sydenham's' choreaPolyarthritis signsErythema marginatumCarditis - (Endo/myo/pericarditis)Subcutaneous nodules

[SPECS]Subcutaneous nodulesRarely seen and when presentUsually associated with severe carditis. Painless, firm, movable, measuring around 0.5 to 2 cm. Located over extensor surfaces of the joints, particularly knees, wrists and elbows

Erythema Marginatum

Erythematous lesions with pale centers and rounded or serpiginous margins.DiagnosisMinor criteria:

PyrexiaECG changes - prolonged PR interval (not if carditis is a major criterion)Arthralgia not if arthritis is a major criteria factor)Raised ESR or CRPHx of previous RF or rheumatic heart disease

[PEACH] The Jones Criteria for Rheumatic Fever, Updated 1992 Major CriteriaCarditisMigratory polyarthritisSydenham's choreSubcutaneous nodulesErythema marginatumMinor CriteriaClinicalfeverArthralgiaLaboratory Elevated acute phase reactants Prolonged PR intervalplusSupporting evidence of a recent group A streptococcal infectionpositive throat culture or rapid antigen detection test; and/ or elevated or increasing streptococcal antibody test(e.g., anti-streptolysin O, anti-deoxyribonuclease B, anti-hyaluronidase).

Maximizing Heart Failure Care: Opportunities to Improve Patient OutcomesPatient Case Scenarios

Slide 2626

ManagementBed-rest until normal CRP for 2 weeks (may take several months)Benzylpenicillin 600mg IM stat Then penicillin V 250 mg/6h p.o.Analgesia for carditis & arthralgia - NSAID e.g. aspirin 90 mg/kg/dayImmobilise joints in severe arthritisHaliperidol 0.5mg/8h p.o. for the chorea(Steroids are not thought to have major benefit., but may improve symptoms).Prophylaxisafter RF prophylaxis involves Penicillin V until aged 25, and then antibiotics before dental surgery etc.