Acute rheumatic fever

ACUTE RHEUMATIC

FEVER

INTRODUCTION

Acute rheumatic fever is an immunological disorder initiated by group A beta hemolytic streptococcus

Antibodies produced against some streptococcus cell wall proteins and sugars react with the connective tissue and heart and result in rheumatic fever

EPIDEMIOLOGY

It constitutes 17 – 50 % of all cardiac patients in hospital

Prevalence rate : 0.55 – 0.67 /1000Incidence rate : 5.3 / 1000 ( ICMR

survey 2010)Age : 5 to 15 yearsBoth sex are equally affected but mitral valve

disease and chorea are common in female , and aortic valve involvement is more in male

PREDISPOSING FACTORS

Poor socioeconomic statusOvercrowdingUnder and poor nutritionFamily history of rheumatic diseaseAge group 5 – 15 yrs ( peak incidence at 8

yrs)

ETIOPATHOGENESIS

Etiology is unknownStrong association with beta hemolytic

streptococcus is indicated by : History of preceding sore throat in 50% of

cases Epidemics of streptococcus infection are

followed by higher incedence Seasonal variation of both are identical Penicillin prophylaxis prevents recurrence > 85% patients show eleveted anti

streptococcal antibody titer

Following streptococcal sore throat there is latent period of 10 days to several weeks

Streptococci have never isolated from rheumatic lesions in joints, heart or blood stream

Streptococcal products against which antibodies produced are streptolysin , hyluronidase , erythrogenic toxins , deoxyribonuclease

Association with HLA – DR3 and B cell antigen serum 883

Patient of rheumatic fever produce antibody against streptococcal cell wall and membrane protiens

Streptococcal antigen and human myocarium appears to be identical antigenically

These antibodies react with human connective tissue mainly cardiac , striated and vascular smooth muscle

Immunoflurescent techniques – antibodies atteched to sarcolemma of cardiac muscle

Streptococcus has hyaluronic acid capsule that prevents phagocytosis

N-acetyl glucosamine is component of cell wall carbohydrate which is immunologically active

That is also present in human connective tissue

N-acetyl glucosamine cross react with antiserum against human connective tissue

DIAGNOSIS CRITERIA

CARDITIS

Pancarditis50 – 60 % of patientsEarly manifestation , around 80 % of patients

developed carditis in first 2 weeksPERICARDITIS :Present in 15 % patients of carditisSevere precordial painFriction rubECG : ST and T changes

MYOCARDITIS :Cardiac enlargementSoft first heart soundProtodiastolic gallopCCFCarey coomb’s murmur

ENDOCARDITIS :Pansystolic murmur of MR and AR

POLYARTHRITIS

Flitting & fleeting migratory polyarthritis, involving major joints

Commonly involved large joints-knee, ankle, elbow & wrist

Occur in 80%In children below 5 yrs arthritis usually mild Arthritis do not progress to chronic diseaseRheumatic joints are generally hot, red,

swollen, and exquisitely tender.

A dramatic response to even small doses of salicylates .

The absence of such a response should suggest an alternative diagnosis.

Rheumatic arthritis is typically not deforming.

Arthritis is the earliest manifestation of acute rheumatic fever.

ERYTHEMA MARGINATUM

Nonpruritic serpiginous or annular erythematous rash more prominent on the trunk & inner proximal portions of the extremities.

Rash is faintly reddish, not raised above the skin and non itching

Rash disappears on exposure to cold & reappears after hot shower.

SUBCUTANEOUS NODULES

Hard, painless, nonpruritic, freely mobile, 0.2 to 2cm in diameter.

Found symmetrically, single or in clusters, on extensor surfaces of both large & small joints, over the scalp or along the spine.

Lasts for weeks.Always associated with severe carditis

SYDENHAM’S CHOREA

Neuropsychiatric disorder10 – 15 % of patientsMore often in pre-pubertal girls than in boys.Characterized by involuntary movements

specially of the face and limbs, muscle weakness, disturbances of speech and gait, poor scholastic performance

Neurologic Signs : Choric Movement & HypotoniaPsychiatric Signs : Emotional Liability,

Hyperactivity, Separation Anxiety, Obsessions & Compulsions

Exceptions to the Jone’s criteria :

Chorea may occur as the only manifestationIndolent carditis may be the only

manifestation if patient come to medical attentio after months of onset

Patients with rheumatic fever recurrence may not fulfill the jone’s criteria

OTHER CLINICAL FEATURES

Abdominal painRapid sleeping HRTachycardia out of proportion of feverMalaiseAnemiaEpistaxisPrecordial pain

DIFFERENTIAL DIAGNOSIS

Juvenile rheumatoid arthritis

Collagen vascular diseases

Virus associated acute arthritis

Hematologic disorder

CLINICAL COURSE

Carditis can cause permanent cardiac damage, signs of mild carditis disappear in weeks but in severe carditis it may last for 6 months

Arthritis subsides within a few days to weeks without treatment

Chorea gradually subsides in 6 to 7 months and usually does not cause permanent neurologic sequelae

MANAGEMENT

Investigation : CBC Acute phase reactant Throat culture ASO titer Chest x ray ECG 2D echo

BED REST

ARTHRITIS ALONE

MILD CARDITIS

MODERATE CARDITIS

SEVERE CARDITIS

BED REST 1-2 wk 3-4 wk 4-6 wk As long as CCF present

INDOOR AMBULATIO

N

1-2 wk 3-4 wk 4-6 wk 2-3 months

ANTIBIOTICS

Benzathine penicillin G 0.6 to 1.2 million units IM

This serves as first dose of penicillin prophylaxis

In patients allergic to penicilline : Erythromycine 40 mg/kg/day in 2 to 4 doses for 10 days

ANTI INFLAMMATORY AGENTS

mild to moderate carditis : aspirine 90-100 mg/kg/day in 4 to 6 divided doses for 4 to 8 weeks, after improvement therapy is withdrawn over 4 to 6 weeks

Arthritis : aspirin is continued for 2 weeks and gradually withdrawn over 2 to 3 weeks

Severe carditis : prednisone 2 mg/kg/day in four divided doses for 2 to 6 weeks

Anti inflammatory agents

ARTHRITIS ALONE

MILD CARDITIS

MODERATE CARDITIS

SEVERE CARDITIS

PREDNISONE

- - - 2 – 6 wk

ASPIRIN 1 – 2 wk 3 – 4 wk 6 – 8 wk 2 – 4 momths

TREATMENT OF CHF

Complete bed rest with orthopneic positionMoist and cool oxygenPrednisoneDigoxineFurosemide if indicated

MANAGEMENT OF SYDENHM’S CHOREA

Reduce physical and emotional stressPhenobarbital ( 15 – 30 mg every 6 to 8 hrs)Haloperidol ( 2 mg every 8 hrs )Valproic acidChlorpromazineDiazepamSteroidsPlasma exchange and IVIG

PROGNOSIS

The more sever the cardiac involvement at the time the patient first seen, greater the incidence of residual heart disease.

The severity of valvular involvement increases with each recurrence.

Valvular disease resolve more frequently when prophylaxis is followed.

PREVENTION

PRIMARY PREVENTION :10 days course of penicillin therapy for

streptococcus pharyngitisNot possible in all patients :

30% patients have subclinical phryngitis 30% patients developed rheumatic fever

without symptoms of streptococcal pharyngitis

SECONDARY PREVENTION :Benzathine penicillin G 1.2 million units IM

every 28 daysOral penicillin V 250 mg BDOral sulfadiazine 1 g or sulfisoxazole 0.5 g

dailyOral erythromycin ethyl succinate 250 mg BD

Recommended duration of prophylaxis :

CATEGORY DURATION

Rheumatic fever without carditis At least 5 yrs or until 21 yrs, whichever is longer

Rheumatic fever with carditisBut without residual heart disease

At least 10 yr or well into adulthood, whichever is longer

Rheumatic fever with carditisBut with residual heart disease

At least 10 yr since last episode and at least up to 40 yr , sometime lifelong

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