Acute Rhematic Fever

Acute Rhematic Fever

Etiology

• Acute rheumatic fever is a systemic disease of childhood,often recurrent that follows group A beta hemolytic streptococcal infection
• 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

·         Rare <3 yrs

·         Girls>boys

·         Common in 3rd world countries

·         Environmental factors--       over crowding, poor sanitation, poverty,malnutrition

·         Incidence more during fall ,winter & early spring

Pathogenesis

l          Delayed immune response to infection with group.A beta hemolytic streptococci.

l          After a latent period of 1-3 weeks, antibody induced immunological damage  occur to heart valves,joints, subcutaneous tissue & basal ganglia of brain.

Pathologic Lesions

l          Fibrinoid degeneration of connective tissue,inflammatory edema, inflammatory cell infiltration &  proliferation of specific cells resulting in formation of  Ashcoff nodules, resulting in-

                                -Pancarditis in the heart

                                -Arthritis in the joints

                                -Ashcoff nodules in the subcutaneous                    tissue

                                -Basal gangliar lesions resulting in                            chorea

Clinical Features

1.Arthritis

·         Migratory polyarthritis, involving major joints

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

·         Occur in 80%,involved joints are exquisitely tender

2.Carditis

·         Manifest as pancarditis(endocarditis, myocarditis and pericarditis

·         Valvulitis occur in acute phase

·         Chronic phase- fibrosis,calcification & stenosis of heart valves.

3.Sydenham Chorea

·         May appear even 6 months after the attack of rheumatic fever

·         Clinically manifest as-clumsiness, deterioration of handwriting, emotional lability

·         Choreiform movements : grimacing of face, abnormal purposeless movements

·         Clinical signs- pronator sign, jack in the box sign , milking sign of hands

4.Erythema Marginatum

·         Unique skin rash

·         Pale center with red irregular margin

·         More on trunks & limbs & non-itchy

5.Subcutaneous nodules

·         Painless,pea-sized,palpable nodules

·         Mainly over extensor surfaces of joints,knee , elbow, spine,scapulae & scalp

Other features (Minor features)

·         Fever-(upto 101 degree F)

·         Arthralgia

·         Pallor

·         Anorexia

·         Loss of weight

Investigations

Laboratory Findings

• High ESR
• CBC: Anemia, leucocytosis
•   Elevated C-reactive protien
•  ASO titre raised
• Throat culture-GABHstreptococci

• ECG- prolonged PR interval
•  Echo cardiography- valve edema,mitral regurgitation,pericardial effusion,decreased contractility
• CXR

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

D/D

1. Rheumatoid arthritis
2. Osteomyelitis
3. Endocarditis
4. SLE

Complicatons

1. CCF
2. Arrhythmias
3. Pericarditis with effusion
4. Valvular heart disease

Management

1. Supportive / symptomatic treatment

1. Bed rest : till acute phase subsides
2. Arthritis : Tab. Aspirin 100mg/kg/day for 2 weeks then 75mg/kg/day for 4-6 weeks in 6 divided doses
3. Carditis and arthritis :Prednisolone 1-2 mg /kg/day in 4 divided doses for 2 weeks and gradually taper. Add aspirin while tapering.
4. Treatment of heart failure

2. Eradication of streptococci

• Inj Benzathine penicillin 1.2 MU IM once
• For penicillin allergy : oral erythromycin 250 mg every 6 hourly for 10 days

3. Prevention of rheumatic fever

1. Primary prevention :

 prevention before the occurrence  of rheumatic fever by early identification and treatment of streptococcal throat infection with penicillin.

b. Secondary prevention

Prevention of recurrence of rheumatic fever.

Inj benzathine penicillin 1.2 MU IM every 3 weeks for 5 years or up to the age of 18 years whichever is longer.(if weight <30 kg 0.6 MU )

Oral penicillin V 250 mg bd every day

Penicillin allergy : erythromycin