Rheumatic Fever / Rheumatic heart disease

DEFINITION :

                     Rheumatic Fever is defined as a delayed inflammatory complication of group A β­ – hemolytic streptococcal (GAS) Pharyngitis that usually occurs within 2 – 4 weeks of acute infection.

ETIOLOGY :                 

• It is causes by Group A Beta- hemolytic streptococcus (Streptococcus pyogenes ) which cause throat infection.
• If it is untreated it leads to Rheumatic fever as a sequela in some risk peoples

          

RISK FACTORS : 

• Not all peoples suffered from throat infection ( Strep throat ) developes Rheumatic fever

• ~3% patient form Rheumatic fever after strep throat
• there are some risk group they are:

                       Children are more likely 

affected

                       Peoples in area of  Poverty & crowding

EPIDIMIOLOY :

                   Peak incidence : 5 – 15 years

TYPES:

1.Acute rheumatic fever :

                  It leads to Pancarditis as a sequela of GAS (group A beta -hemolytic streptococcus infection

2.Chronic rheumatic fever :

                  It can lead to chronic valvular changes due to the complications of Acute rheumatic fever

PATHOGENESIS :

                

CLINICAL FEATURES :

1) Joints – Migratory Polyarthritis

        Migratory - One after another

        Poly - Multiple

        Arthritis - Large joint inflammation

2) heart :

Pancarditis - all three layers of heart inflammation

• Endocarditis - inflammation of endocardium (innerlying)
• Myocarditis – inflammation of myocardium

                   inflammed area in myocardial tissue are called ASCHOFF BODY (area of fibrinoid necrosis) which contain immune cells and ANTISCHKOW CELLS (enlarged macrophages with caterpillar like cells )

                   myocarditis is the leading cause of death in Rheumatic fever because inflammation cause heart wall unable to contract – heart failure

• Pericarditis – inflammation of outer covering of heart

                             cause pain and friction rub

Valvular lesions :

• Mitral valve – 65% of case

               Early : Mitral regurgitation or prolapsed

               Late : Mitral stenosis

• Aortic valve – 25% of case

             Aortic regurgitation

             Aortic stenosis

• Tricuspid valve - 10%

3)    Subcutaneous Nodules :

Firm lump under skin made of collagen 

4)    Erythema Marginatum :

Reddish rash shows like rings in arms and in trunk

5)    Sydenhom chorea

• rapid movements in face & arm
• Due to autoimmune reaction in basal ganglia
• Occurs late atleast 3 months after infection

DIAGNOSIS :

                   Diagnosis is based on Jones criteria.

Jones criteria :

          Interpretations : ( two major or one minor + two minor ) are required for diagnosis.

MAJOR CRITERIA :

• Arthritis – Migratory Polyarthritis
• Carditis – pancarditis, including valvulitis
• Sydenhem chorea – CNS involvement
• Subcutaneous nodules
• Erythema marginatum

MINOR CRITERIA :

• Arthralgia – joint paint
• Fever
• Increased ESR, CRP ( acute phase reactants )
• ECG – prolonged PR interval

TREATMENT:

1)    General measures : 

bed rest ( especially in patient with carditis )

2 )Antibiotics : to eradicate GAS

Drug of choice : oral penicillin V ( beta lactum antibiotics )

 :Alternatives :

                           Amoxicillin

 Benzathine 

 Cephalosporins

 Macrolides

3)    Therapy for arthritis and fever :

NSAIDS – Non-Steroidal Anti Inflammatory Drugs

Preferred : salicylates ( eg : asprin )

Childrens : ibuprofen, naprozen

Glucocorticoids ( if  NSAIDS  fails )

4)    Therapy for heart failure :

                      Diuretics and conventional theraphy

5)    Therapy for myocarditis :

Monitoring and treatment for arrhythmias ( amiodarone )

6)    Damage to cardiac valves :

Surgery or interventional reconstructive measures may be considered

atleast one year after acute inflammatory phase

PROGNOSIS :

                   Joints – not permentely affected

                   Heart – permentently affected

RHEUMATIC FEVER LICKS 

THE JOINT 

BUT BITES THE HEART

                   Early death in rheumatic fever is usually due to myocarditis rather than valvular defects.

PREVENTION :

1)    Primary Prevention : Penicillin V

2)    Secondary Prevention :

a.     Antibiotics prophylaxis to prevent recurrence

b.     Drug of choice : 1m Penicillin G benzathine

                                                             i.      In patient with Penicillin allergy : oral marcolides

                                                           ii.      Usually administered every 28 days

c.      Immediately follows antibiotic treatment of acute rheumatic fever

d.     Duration depends on risk and severity of original episodes

Duration :

Rheumatic fever without carditis :

          5 years or until the patient age 21

     Rheumatic fever with carditis :

          10 years or until the patient age 21

    Rheumatic fever with carditis and permanent valvular heart defects :

          10 years or until age 40