Cardiovascular system CVS

ACUTE RHEUMATIC FEVER

ACUTE RHEUMATIC FEVER
Multisystem disorder typicaly follows an episode f strep.pharyngitis(2-3 weeks) and usually presents with fever ,anorexia ,lethargy and joint pains. Autoimmune consequence of infection (pharyngeal infection not the skin infection) with Group A beta haemolytic streptococcal infection Generalized inflammatory response affecting brains, joints, skin, subcutaneous tissues & the heart

ACUTE RHEUMATIC FEVER

Supporting evidences: About 66% of the patients with an acute episode of rheumatic fever have a history of an upper respiratory tract infection 2-3 weeks before The peak age (6-15 yrs) & seasonal incidence of acute rheumatic fever closely parallel those of GABHS infections

ACUTE RHEUMATIC FEVER

Predisposing factors: Family history of rheumatic fever Low socioeconomic status (poverty, poor hygiene, medical deprivation) Age: 5-15 years

Very rare in developed counteries while still endemic in developing counteries. Histologically ,fibrinoid degeneration in collagen CT Aschoff nodules are pathognomonic ,only in the heart(multinucleatd gaint cells surronded by macrophage and T lymphocytes)

CLINICAL MANIFESTATIONS

No pathognomonic clinical or laboratory finding for acute rheumatic fever Duckett Jones in 1944 proposed guidelines to aid in diagnosis & to limit overdiagnosis Jones criteria for the diagnosis of acute rheumatic fever 2 major criteria or 1 major & 2 minor criteria pluse supporting evidence (microbiologic or serologic) of recent GABHS infection



DIAGNOSIS(JONES CRITERIA)
MAJOR MANIFESTATIONS
MINOR MANIFESTATIONS
SUPPORTING EVIDENCE OF ANTECEDENT GROUP A STREPTOCOCCAL INFECTION******
Carditis
Clinical features:Arthralgia Fever Previous rheumatic fever
-Elevated or increasing streptococcal antibody titer(Antistreptolysin O) -Positive throat culture or rapid streptococcal antigen test or streptococcal sore throat or recent scarlet fever)
Polyarthritis
Laboratory features:Elevated acute phase reactants: ESR, C-reactive protein Prolonged PR interval Leucocytosis
Erythema marginatum
Subcutaneous nodules
Chorea

MAJOR MANIFESTATIONS

Arthritis
Most common (75%) Usually an early feature Involves larger joints: the knees, ankles, wrists & elbows Rheumatic joints: hot, red, swollen & exquisitely tender (friction of bedclothes is uncomfortable The joint involvement is characteristically migratory in nature A dramatic response to even small doses of salicylates is another characteristic feature of the arthritis

Carditis

Carditis & chronic rheumatic heart disease: most serious manifestations of acute rheumatic feve Occurs in 50% of patients pancarditis with active inflammation of myocardium, pericardium & endocardium May manifest as SOB,palpitations or chest pain. Other features , tachycardia out of proportion to fever, cardiac enlargment & cardiac murmurs(soft systolic murmur ,soft mid diastolic murmur Carey coombs), aortic regurgitation in 50%, other valves rarely involved.


Carditis
Pericarditis may cause chest pain ,pericardial rub. ECG changes are common ST,T wave ,conduction defects. Echocardiographic findings: pericardial effusion, decreased ventricular contractility & aortic &/or mitral regurgitation The major consequence of acute rheumatic carditis is chronic, progressive valvular disease

Chorea

Sydenham chorea: one third of patients with acute rheumatic fever More in females A long latency period (1-6 mo) between streptococcal pharyngitis & the onset of chorea Begins with emotional lability & personality changes (poor school performance). Followed in 1-4 weeks by characteristic spontaneous, purposeless involentery chorea movement of the hands ,feet or face (lasts 4-8 months) followed by motor weakness Recovery within few monthes ,1/4 will develop chronic rheumatic valve disease.

Erythema Marginatum

A rare (<5% of patients with acute rheumatic fever) but characteristic rash of acute rheumatic fever It consists of erythematous, serpiginous, macular lesions with pale centers that are not pruritic It occurs primarily on the trunk & extremities, not on the face & it can be accentuated by warming the skin

Subcutaneous Nodules

MINOR MANIFESTATIONS

MINOR MANIFESTATIONS

Clinical:1. Arthralgia (in the absence of polyarthritis as a major criterion) 2. Fever (typically temperature ≥102°F & occurring early in the course of illness)Previous rheumatic feverLaboratory minor manifestations:1.Elevated acute-phase reactants (C-reactive protein, erythrocyte sedimentation rate, polymorphonuclear leukocytosis) 2. Prolonged PR interval on electrocardiogram (1st degree heart block)

ESSENTIAL CRITERIA An absolute requirement for the diagnosis of acute rheumatic fever is supporting evidence of a recent GABHS infection

DIFFERENTIAL DIAGNOSIS

ARTHRITIS
Rheumatoid arthritis
Reactive arthritis (Shigella, Salmonella, Yersinia)
Serum sickness
Sickle cell disease
Malignancy
Systemic lupus erythematosus
Lyme disease (Borrelia burgdorferi)
Gonococcal infection (N.gnorrhoeae)

TREATMENT

Bed rest :reduce joint pains and cardiac workload, till symptoms and markers of inflammation settled Antibiotic Therapy: 10 days of orally administered penicillin or erythromycin or a single intramuscular injection of benzathine penicillin to eradicate GABHS from the upper respiratory tract Afterwards, the patient should be started on long-term antibiotic prophylaxis

TREATMENT

TREATMENT

TREATMENT

Supportive therapies for patients with moderate to severe carditis include digoxin, fluid & salt restriction, diuretics & oxygen The cardiac toxicity of digoxin is enhanced with myocarditis

PREVENTION

SECONDARY PREVENTION
For how long?
CATEGORY
DURATION
Rheumatic fever without carditis
At least for 5 yr or until age 21 year, whichever is longer
Rheumatic fever with carditis but without residual heart disease (no valvular disease)
At least for 10 yr or well into adulthood, whichever is longer
Rheumatic fever with carditis & residual heart disease (persistent valvular disease)
At least 10 yr since last episode & at least until age 40 yr; sometime lifelong

SECONDARY PREVENTION

What method of prophylaxis should be used?
DRUG
DOSE
ROUTE
Penicillin G benzathine
600,000 U for children, ≤27 kg1.2 million U for children >27 kg, every 4 wk Intramuscular
OR
Penicillin V


Oral
OR
Sulfadiazine or sulfisoxazole
0.5 g, once a day for patients ≤60 lb; 1.0 g, once a day for patients >60 lb Oral