ACUTE RHEUMATIC FEVER

DR Mohamad Jarrah Assistant Professor Cardiologist JUST

Definition
An acute systemic immune disease that may develop after an infection with Group A betahemolytic Streptococcal infection of the pharynx. This disease can affect the HEART, JOINTS, SKIN, SUBCUTANEOUS TISSUE, BRAIN, RESPIRATORY SYSTEM, VESSELS, SEROSAL MEMBRANES, TENDONS AND FASCIAL SHEATHS

GENERAL CONSIDERATIONS
Usually preceded 2-3 weeks (1-5 weeks) by sore throat. Peak incidence 5- 15 years. Rare in <4 year olds and > 40 years 3% of pt dev ARF

MODIFIED JONES CRITERIA

MAJOR: Polyarthritis Carditis Chorea Subcutaneous nodules Erythema marginatum

MINOR CRITERIA
Clinical Fever Polyarthralgia h/o previous ARF or Rheum. heart disease Lab Reversible prolongation of PR interval Inc ESR Inc C Reactive Protein + throat culture Or rapid streptococcal antigen test Inc ASO titre

POLYARTHRITIS
Migratory flitting and fleeting Involves large joints sequentially Polyarthritis- in adults only a single joint may be affected Lasts 1-5 weeks Occurs in 75% or patients Subsides without residual deformity Dramatic response of arthritis to therapeutic doses of aspirin or NSAIDs

CARDITIS
1. Most likely in children and adolescents Occurs in 1/3 of cases Any of the following signs suggest the presence of carditis Endocardial- MR or AR murmurs indicative of dilatation of valve ring with or without associated valvulitis -Short mid-diastolic murmur (Carey-Coombs) may be present - Changing quality of heart sounds 2. Myocardial - Tachycardia even at rest. Arrhythmias or ectopic beats - Cardiomegaly- on physical exam, CXR or ECHO - Congestive cardiac failure right or left sided 3. Pericardial - Pericarditis - Pericardial effusion ECG Changes - Changing contour of P waves - Inversion of T waves - Prolongation of PR interval Maybe self limiting or may lead to slowly progressive valvular deformity Mitral valve attacked in 75% cases, aortic in 30% ( but rarely as the sole valve), tricuspid and pulmonary in < 5% cases

SYDENHAMS CHOREA
Involuntary choreo- athetoid movements primarily of the face, tongue, and upper extremities Maybe sole manifestation- in 50% of cases no other signs of RF Girls more frequenty affected Rare in adults Lease common(<3%) but most diagnostic of the manifestations of RF

Erythema Marginatum
Rapidly enlarging macules that assume the shape of rings or crescents with clear centres They may be raised, confluent and either transient or persistent.

Subcutaneous Nodule
Uncommon except in children Small (<2cm in diameter) firm & nontender Attached to fascia, or tendon sheaths over bony prominences Persist for days or weeks Are recurrent Indistinguishable from rheumatoid nodules

Also there features: Pneumonia Epistaxis Erythema nodosum Abdominal pain

REQUIRED FOR DIAGNOSIS

Two major criteria OR One major and two minor criteria

DIFFERENTIAL DIAGNOSIS
Rheumatoid arthritis Osteomyelitis Endocarditis Chronic meningiococcemia SLE Lyme disease Sickle cell disease Surgical abdomen

TREATMENT
PHARYNGITIS Benzathene penicillin 1.2 million units ( 50,000 units/kg to a max of 1.2 million units) is injected IM once or Inj Procaine penicillin 600,000 units once daily for 10 days Erythromycin can be substituted ( 40mg/kg/day) CARDITIS Bed rest until temp, ESR, resting pulse rate and ECG have all returned to normal Prednisone if there is CCF or cardiomegaly POLYARTHRITIS Anti inflammatory agent - Aspirin markedly reduces fever, joint pain and swelling No effect on the natural course of the disease 100mg / kg/day in 4-6 divided doses. Can be reduced to 75mg/Kg/day once there is a response . Given for 4-6 weeks Toxicity includes- tinnitus, vomiting and GI bleeding. When response to aspirin is inadequate a short course of prednisone (1 mg/kg/day) orally daily usually causes rapid improvement of joint symptoms. It is tapered over 2 weeks. Add aspirin when tapering begins.

PREVENON OF ARF-PRIMARY
Early and adequate treatment of Strep. throat infections with a penicillin or Azithromycin will prevent Rheumatic Fever Avoidance of overcrowding & improved hygiene will decrease the incidence of pharyngitis

PREVENTION -SECONDARY
Those who have had RF can have recurrences Recurrences are most common in children and in those patients who have had carditis during their initial episode of RF Recurrences are prevented by giving Benzathine penicillin 1.2million units IM every 4 week OR Oral penicillin 250 mg bid Erythromycin 250 mg bid Azithromycin Duration controversial: 5 years after last attack or at 25 years, whichever is later (earlier recommendation: life-long) Those with cardiac involvement and in high risk group- military personnel, health staff, school teachers, parents of young children- life long prophylaxis

IMPORTANT!! The complication of untreated, or inadequately treated Acute rheumatic fever is RHEUMATIC HEART DISEASE

RHEUMATIC HEART DISEASE

Results from single or repeated attacks of RF Rigidity and deformity of valves resulting in stenosis or incompetence or both Mitral valve alone in 50% Mitral + Aortic in 25% Pure aortic uncommon History of RF obtained in 60% Should receive prophylatic penicillin monthlyand preceding dental extractions,urologic and surgical procedures to prevent endocarditis