Date post: | 21-May-2015 |
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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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