Objectives

       
Etiology Epidemiology Pathogenesis Pathologic lesions Clinical manifestations & Laboratory findings Diagnosis & Differential diagnosis Treatment & Prevention Prognosis

Etiology 
Acute rheumatic fever is a systemic disease of
childhood,often recurrent that follows group A beta hemolytic streptococcal infection  It is a delayed non-suppurative sequelae to nonURTI with GABH streptococci.  It is a diffuse inflammatory disease of connective tissue,primarily involving heart,blood vessels,joints, subcut.tissue and CNS

Epidemiology 
Ages 5-15 yrs are most susceptible 5 Rare <3 yrs  Girls>boys  Common in 3rd world countries  Environmental factors-- over factors-crowding, poor sanitation, poverty,  Incidence more during fall ,winter & early spring

Pathogenesis 
Delayed immune response to infection
with group.A beta hemolytic streptococci.  After a latent period of 1-3 weeks, 1antibody induced immunological damage occur to heart valves,joints, subcutaneous tissue & basal ganglia of brain

Group A Beta Hemolytic Streptococcus 

Strains that produces rheumatic fever M types l, 3, 5, 6,18 & 24  Pharyngitis- produced by GABHS can Pharyngitislead to- acute rheumatic fever , torheumatic heart disease & post strept. Glomerulonepritis  Skin infection- produced by GABHS leads infectionto post streptococcal glomerulo nephritis only. It will not result in Rh.Fever or carditis as skin lipid cholesterol inhibit antigenicity

Diagrammatic structure of the group A beta hemolytic streptococcus
Antigen of outer protein cell wall of GABHS Cell wall induces antibody Protein antigens response in victim which Group carbohydrate result in autoimmune Peptidoglycan damage to heart valves, Cyto.membrane sub cutaneous tissue,tendons, Cytoplasm joints & basal ganglia of brain
Capsule

««««««««««««««««« ««...

Pathologic Lesions 
Fibrinoid degeneration of connective tissue,
inflammatory edema, inflammatory cell infiltration & proliferation of specific cells resulting in formation of Ashcoff nodules, resulting inin-Pancarditis in the heart -Arthritis in the joints -Ashcoff nodules in the subcutaneous tissue -Basal gangliar lesions resulting in chorea 

  

Rheumatic Carditis Histology (40X)

Histology of Myocardium in Rheumatic Carditis (200X)

1.Arthritis

Clinical Features 

Flitting & fleeting migratory polyarthritis,     
involving major joints Commonly involved jointsjointsknee,ankle,elbow & wrist Occur in 80%,involved joints are exquisitely tender In children below 5 yrs arthritis usually mild but carditis more prominent "RF licks the joints and bites the heart" Arthritis do not progress to chronic disease

Clinical Features (Contd)
2.Carditis 
Manifest as pancarditis(endocarditis, pancarditis(endocarditis,
myocarditis and pericarditis),occur in 40-50% of 40cases  Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ  Valvulitis occur in acute phase  Chronic phase- fibrosis,calcification & stenosis phaseof heart valves(fishmouth valves) 

Auscultatory findings are the most conclusive.  First heart sound, if indistinct or muffled
suggests first degree heart block.  Both sounds may be indistinct if pericardial effusion is present. There is TIC-TAC rhythm. TIC A pericardial rub (scratchy, leathery sound altered by varying pressure of stethoscope) is heard in both phases of cardiac cycle. If it varies with respiration, then it may be of pleuropericardial origin.

An apical, high pitched, blowing, holosystolic murmur, of at least grade II/VI indicates mitral valvulitis. It may be accompanied by a low pitched mid diastolic murmur (Carey Coomb's murmur) which occurs due to relative stenosis of mitral valve in relation to a dilated left ventricle and large blood flow in early diastole. There is no presystolic accentuation or opening snap. Appearance of a new murmur or changing quality of murmur indicates presence of carditis.

Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae

Another view of thick and fused mitral valves in Rheumatic heart disease

Clinical Features (Contd)
3.Sydenham Chorea 
Occur in 5-10% of cases 5 Mainly in girls of 1-15 yrs age, male to female ratio of 1:2 1 It is abrupt, purposeless, unilateral or bilateral      
involuntary movement, which disappears during sleep Hemichorea is possible It does not occur in adults It has a long latent period, may appear even 6/12 after the attack of rheumatic fever All voluntary muscles may be involved Clinically manifest as-clumsiness, deterioration of ashandwriting, emotional lability or grimacing of face Clinical signs- pronator sign, jack in the box sign , signsmilking sign of hands

Clinical Features (Contd)
4.Erythema Marginatum 

Occur in <5%.  Unique,transient,serpiginous-looking Unique,transient,serpiginous   
lesions of 1-2 inches in size 1Pale center with red irregular margin More on trunks & limbs & non-itchy, facial nonsparing Worsens with application of heat Often associated with chronic carditis

Clinical Features (Contd) 5.Subcutaneous nodules 
Occur in 10%  They occur after first few weeks of illness  Firm, painless, pea-sized, palpable peanodules  Mainly over extensor surfaces of joints,spine,scapulae & scalp  Associated with strong seropositivity  Always associated with severe carditis

Clinical Features (Contd)
Other features (Minor features) 

Fever-(upto 101 degree F) Fever Arthralgia  Pallor  Anorexia  Loss of weight

Laboratory Findings 
High ESR  Anemia, leucocytosis  Elevated C-reactive protien C ASO titre >200 Todd units.

(Peak value attained at 3 weeks,then comes down to normal by 6 weeks)  Anti-DNAse B test Anti Throat culture-GABHstreptococci culture-

Laboratory Findings (Contd) 
ECG- prolonged PR interval, 2nd or 3rd ECGdegree blocks, ST-depression, TSTTinversion  2D Echo cardiography- valve edema,mitral cardiographyregurgitation, LA & LV dilatation, pericardial effusion, decreased contractility

Diagnosis 
Rheumatic fever is mainly a clinical
diagnosis  No single diagnostic sign or specific laboratory test available for diagnosis  Diagnosis based on MODIFIED JONES CRITERIA

Jones Criteria (Revised) for Guidance in the Diagnosis of Rheumatic Fever*
Major Manifestation
Carditis Polyarthritis Chorea Erythema Marginatum Subcutaneous Nodules

Minor Manifestations
Clinical Previous rheumatic fever or rheumatic heart disease Arthralgia Fever Laboratory Acute phase reactants: Erythrocyte sedimentation rate, C-reactive protein, leukocytosis Prolonged PR interval

Supporting Evidence of Streptococal Infection
Increased Titer of AntiStreptococcal Antibodies ASO (anti-streptolysin O),

others
Positive Throat Culture for Group A Streptococcus Recent Scarlet Fever

*The presence of two major criteria, or of one major and two minor criteria, indicates a high probability of acute rheumatic fever, if supported by evidence of Group A streptococcal nfection.

Exceptions to Jones Criteria
Chorea alone, if other causes have been excluded Insidious or late-onset carditis with no lateother explanation Patients with documented RHD or prior rheumatic fever,one major criterion,or of fever,arthralgia or high CRP suggests recurrence

Differential Diagnosis 
Juvenile rheumatiod arthritis  Septic arthritis  Sickle-cell arthropathy Sickle Kawasaki disease  Myocarditis  Scarlet fever  Leukemia

Treatment 
Step I - primary prevention
(eradication of streptococci)  Step II - anti inflammatory treatment (aspirin,steroids)  Step III- supportive management & IIImanagement of complications  Step IV- secondary prevention IV(prevention of recurrent attacks)

STEP I: Primary Prevention of Rheumatic Fever
(Treatment of Streptococcal Tonsillopharyngitis)
Agent Benzathine penicillin G Dose Mode Duration Once 600 000 U for patients Intramuscular 27 kg (60 lb) 1 200 000 U for patients >27 kg

or
Penicillin V Children: 250 mg 2-3 times daily Oral (phenoxymethyl penicillin) Adolescents and adults: 500 mg 2-3 times daily For individuals allergic to penicillin Erythromycin: 20-40 mg/kg/d 2-4 times daily (maximum 1 g/d) Oral 10 d Estolate 10 d

or
Ethylsuccinate 40 mg/kg/d 2-4 times daily (maximum 1 g/d) Oral 10 d

Recommendations of American Heart Association

Step II: Anti inflammatory treatment
Clinical condition
A r th r itis o n ly

Drugs
A s p ir in 7 5 -1 0 0 m g / k g / d a y , g iv e a s 4 d iv id e d d o s e s f o r 6 w eeks ( A tt a in a b lo o d le v e l 2 0 3 0 m g /d l) P r e d n is o lo n e 2 -2 .5 m g / k g / d a y , g iv e a s tw o d iv id e d d o s e s f o r 2 w eeks T aper over 2 w eeks & w h ile t a p e r in g a d d A s p ir in 7 5 m g /k g / d a y fo r 2 w e e k s . C o n t in u e a s p ir in a lo n e 1 0 0 m g /k g /d a y fo r a n o th e r 4 w e e k s

C a r d itis

3.Step III: Supportive management & management of complications 

Bed rest  Treatment of congestive cardiac failure:
-digitalis,diuretics  Treatment of chorea: -diazepam or haloperidol  Rest to joints & supportive splinting

STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks)
Agent
Benzathine penicillin G

Dose
1 200 000 U every 4 weeks*

Mode
Intramuscular

or
Penicillin V 250 mg twice daily Oral

or
Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral 1.0 g once daily for patients >27 kg (60 lb)

For individuals allergic to penicillin and sulfadiazine
Erythromycin 250 mg twice daily Oral

*In high-risk situations, administration every 3 weeks is justified and recommended

Recommendations of American Heart Association

Duration of Secondary Rheumatic Fever Prophylaxis
Category
Rheumatic fever with carditis and residual heart disease (persistent valvar disease*)

Duration
At least 10 y since last episode and at least until age 40 y, sometimes lifelong prophylaxis 10 y or well into adulthood, whichever is longer

Rheumatic fever with carditis but no residual heart disease (no valvar disease*) Rheumatic fever without carditis

5 y or until age 21 y, whichever is longer

*Clinical or echocardiographic evidence.
Recommendations of American Heart Association

Prognosis 
Rheumatic fever can recur whenever the
individual experience new GABH streptococcal infection,if not on prophylactic medicines  Good prognosis for older age group & if no carditis during the initial attack  Bad prognosis for younger children & those with carditis with valvar lesions

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