Acute rheumatic fever (ARF) is a delayed, nonsuppurative sequela of a pharyngeal infection with the group A streptococcus

Rheumatic fever can occur at any age, although most cases occur in children 5 to 15 years of age

The mean incidence of ARF is 19 per 100,000 In the United States and other developed countries, the incidence of ARF is much lower at 2 to 14 cases per 100,000; this is probably due to improved hygienic standards and routine use of antibiotics for acute pharyngitis

Following the initial pharyngitis, a latent period of two to three weeks occurs before the first signs or symptoms of ARF appear

(GAS) tonsillopharyngitis presents with abrupt onset of sore throat, tonsillar exudate, tender cervical adenopathy, and fever, followed by spontaneous resolution within two to five days.

The disease presents with various manifestations that may include Arthritis Carditis Chorea Subcutaneous nodules Erythema marginatum

EXTRACARDIAC LESIONS OF RHEUMATIC FEVER

Migratory polyarthritis, involving major joints Commonly involved joints-knee,ankle,elbow & wrist Occur in 80%,involved joints are exquisitely tender Arthritis do not progress to chronic disease Arthritis

Joints: Rheumatic arthritis affect the large joints joint inflammation is followed by joint resolution, then another joint become inflamed followed by resolution and so on. The affected joint is painful, tender, hot & swollen

Occur in <5%. Unique,transient,serpiginous-looking lesions of 1-2 inches in size Pale center with red irregular margin More on trunks & limbs & non-itchy Often associated with chronic carditis Erythema Marginatum

Copyright © American College of Rheumatology Slide Collection. All rights reserved. Rheumatic fever: erythema marginatum

Skin: Rheumatic subcutaneous nodules occur over bony prominences and their structure is similar to the Aschoff bodies

Occur in 10% Painless,,palpable nodules Mainly over extensor surfaces of joints,spine,scapulae & scalp Always associated with severe carditis Subcutaneous nodules

Brain: Rheumatic chorea (rapid involuntary purposeless movements); it is due to inflammation of the basal ganglia. The condition is reversible

Occur in 5-10% of cases Mainly in girls of 1-15 yrs age Clinical signs- pronator sign, milking sign of hands Sydenham Chorea

Manifest as pancarditis (endocarditis, myocarditis and pericarditis),occur in 40-50% of cases Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ Valvulitis occur in acute phase Chronic phase- fibrosis,calcification & stenosis of heart valves(fishmouth valves) Carditis

Laboratory Findings High ESR Anemia, leucocytosis Elevated C-reactive protien ASO titre >200 Todd units. (Peak value attained at 3 weeks,then comes down to normal by 6 weeks)

Theories of Pathogenesis: – Toxic products of streptococci – Immunologic cross-reactivity between Streptococcal substances and heart muscle (heart reactive antibodies)

JONES' CRITERIA FOR DIAGNOSIS OF RF: Major Manifestations – Carditis (friction rub, murmur, cardiomegaly, CHF) – Arthritis (migratory polyarthritis, swollen, red, tender) – Chorea – Subcutaneous nodules – Erythema marginatum Minor Manifestations – Clinical Fever Arthralgia History of rheumatic fever or rheumatic heart disease – Laboratory Acute phase reactants (ESR, C-reactive protein, leukocytosis) Prolonged P-R interval on ECG

PATHOLOGY OF RHEUMATIC FEVER Cardiac Disease (Rheumatic heart disease) Extra-Cardiac Disease

RHEUMATIC HEART DISEASE Rheumatic heart disease: all the heart layers are affected (pancarditis) 1.Rheumatic myocarditis 2.Rheumatic pericarditis 3.Rheumatic endocarditis

1- Rheumatic myocarditis: Acute phase: it is characterized by the development of pathognomonic lesions called Aschoff ’ s Bodies within the myocardium. Gross features: Aschoff bodies are multiple tiny nodules (1-2 mm in diameter) Microscopic features: Aschoff body is a lesion composed of: – Fibrinoid necrosis ( destroyed fragmented collagen) – Surrounded by lymphocytes and histiocytes & – Aschoff cells (large mononuclear or multinuclear macrophages)

Chronic phase: Over years or decades the Aschoff bodies undergo fibrous scarring

2- Rheumatic Pericarditis:

3- Rheumatic Endocarditis: It affects both mural and valvular endocardium 1.Mural Endocardium: i- Acute phase: Aschoff bodies develop in the endocardium ii- Chronic phase: healing results in a white patch

Valvular Endocardium – Vegetations (thrombi) develop at the lines of contact of the cusps causing friction of the swollen cusps.

Rheumatic Mitral Valve Small vegetations are formed at injured parts

CHRONIC RHEUMATIC VALVULAR DISEASE Mitral & Aortic Valves Pathology: – Thickening of valve leaflet, especially along the lines of closure – Fusion of commissures – Result is mitral or aortic stenosis, insufficiency, or both

Rheumatic Mitral Stenosis Thick valve leaflet Fusion of commisures

Three major goals of treatment: Symptomatic relief of acute disease manifestations Eradication of the group A beta-hemolytic streptococcus (GAS) Prophylaxis against future GAS infection to prevent recurrent cardiac disease

Oral penicillin V is the agent of choice for treatment of GAS pharyngitis in many clinical settings given its proven efficacy, safety, narrow spectrum, and low cost Amoxicillin is often used in place of oral penicillin in children, since the taste of the amoxicillin suspension is more palatable than that of penicillin First-generation cephalosporins are an acceptable alternative to penicillin and amoxicillin in the setting of treatment failure or beta-lactam hypersensitivity

Duration — In general, the conventional duration of oral antibiotic therapy to achieve maximal pharyngeal GAS eradication rates is 10 days

Injections of benzathine penicillin provide bactericidal levels against GAS for 21 to 28 days