Rheumatic Fever

Ebraheem Samir Hesamudin

Teaching Assistant, Pediatric Department
University of Jeddah
Objectives
• Definition
• Incidence and epidemiology
• Pathophysiology
• Criteria
• Manifestation
• Investigation
• Management
• Prognosis and recurrence
Definition
• systemic illness that may occur following group A beta hemolytic
streptococcal (GABHS) (Streptococcus pyogenes) pharyngitis in
children.
• Believed to result from an autoimmune response; however, the exact
pathogenesis remains unclear.
Incidence and Epidemiology
• Age : school children 5–15 years old, rare < 3 years of age
• Male = Female
• most important form of acquired heart disease in children and young
adults living in resource-limited countries
• The impact of socioeconomic status is illustrated by a study from
Kinshasa where the prevalence based on clinical examination was
22.2 per 1000 among children who lived in a dense poor urban
environment but only 4 per 1000 among children attending the city
schools
• accounts for approximately 15 percent of all patients with heart
failure
Pathophysiology
• Rheumatic fever develops in children and adolescents following
pharyngitis with GABHS.
• Only infections of the pharynx have been shown to initiate or
reactivate rheumatic fever.
• Organisms attach to the epithelial cells of the upper respiratory tract
→ incubation period of 2-4 days → acute inflammatory response
(symptoms) 3-5 days → resolution.
• In a small percent of patients, infection leads to rheumatic fever
several weeks after the sore throat has resolved.
Group A streptococci
• GABHS may cause suppurative diseases (eg, pharyngitis, impetigo,
cellulitis, myositis, pneumonia, sepsis), and associated with
nonsuppurative diseases (e.g. rheumatic fever, acute
poststreptococcal glomerulonephritis)
• Group A streptococci (GAS) elaborate the cytolytic toxins,
streptolysins S and O, Of these 2 toxins, streptolysin O induces
persistently high antibody titers.
• The presence of the M protein is the most important virulence factor
for GAS infection in humans, which is similar to antigens in human
heart.
Detect GAS
• Throat culture
• Throat sample for PCR
• Elevated Anti streptolysin O
• Elevated Anti Dnase B
Treat GAS and prevent from RHD
• Penicillin treatment shortens the clinical course of streptococcal
pharyngitis and more importantly prevents the major sequelae, Even
when started as long as 9 days after the onset of acute illness,
penicillin effectively prevents primary attacks of rheumatic fever.
Diagnosis
Exceptions
• There are two circumstances in which a diagnosis of ARF can be
made without strict adherence to the previous criteria
1. Chorea as the only manifestation.
• These patients should undergo evaluation for carditis with echocardiogram
2. Indolent carditis as the only manifestation in patients who come to
medical attention months after acute GAS infection.
Clinical manifestations
Carditis 50-70%
• often produces a Pancarditis, characterized by endocarditis, myocarditis,
and pericarditis, and is the most serious complication.
• Endocarditis is manifested as Mitral (65-70% of patients) and Aortic (25%)
valve insufficiency
• Severe valve insufficiency during the acute phase may result in congestive
heart failure and even death (1% of patients)
• When pericarditis is present, it rarely affects cardiac function or results in
constrictive pericarditis.
• RHD is responsible for 99% of mitral valve stenosis in adults, and it may be
associated with atrial fibrillation from chronic mitral valve disease and
atrial enlargement
Pericarditis?? Characteristic? Improve? ECG finding?
Pain improve when leaning forward
pericardial friction rub
Arthritis

• most common symptom (70-75%)

Usually polyarthritis, but in high-risk population can be monoarthritic
Arthritis Vs. arthralgia ??
• Begins in the large joints of the lower extremities (ie, knees, ankles)
and migrates to other large joints in the lower or upper extremities
(ie, elbows, wrists).
• reaches maximum severity in 12-24 hours and persists for 2-6 days
(rarely >4 weeks)
• responds rapidly to aspirin and NSAID
Sydenham chorea
• Involuntary, random muscle movements.
• long latency period exists between streptococcal pharyngitis (1-6 mo)
• Usually benign
• Daily handwriting samples can be used as an indicator of progression
or resolution of disease.
• Complete resolution of the symptoms typically occurs, with
improvement in 1-2 weeks and full recovery in 2-3 months
• Erythema marginatum (5-13%)
nonpruritic macules or papules In covered areas and aggravated by
heat
• Subcutaneous nodules (0-8%):
Infrequent manifestation of rheumatic fever
nodules are firm, nontender, and free from attachments to the
overlying skin, and they range from a few millimeters to 1-2 cm
Erythema marginatum Can be associated with :
Sepsis
drug reactions
glomerulonephritis.
Other manifestations
• Fever:
>39°C , no characteristic pattern are present initially in almost every
patient with acute rheumatic fever.
low grade (38-38.5°C) in children with mild carditis or absent in
patients with pure chorea.
fever decreases without antipyretic therapy in approximately 1 week,
but low-grade fevers persist for 2-3 weeks.
• Arthralgias
• High ESR >= 60 (>30 in high-risk patient)
• High CRP >=3 mg/dl
Investigation?
Management
• Primary prevention :
treat GAS with penicillin (or macrolides, or clindamycin, or 1st
generation cephalosporin if allergic to penicillin)
• Treatment of acute illness
• Secondary prevention
Treatment for patients with rheumatic fever
• Treat residual GABHS pharyngitis
• Aspirin in anti-inflammatory doses (80 mg/kg/day) effectively reduces
all manifestations of the disease except chorea, keep for (6-8 wk) then
withdraw gradually
High dose aspirin can cause high liver enzymes
• Chorea can be managed with diazepam
• If moderate to severe carditis is present as indicated by cardiomegaly,
third-degree heart block, or CHF, add PO prednisone to aspirin
therapy for 2-6 weeks with gradual withdraw, while maintaining
aspirin for an additional 2-4 weeks
• Treat heart failure:
Bed rest, fluid and salt restriction
Diuretics and digoxin
Antihypertensive medications
• Surgical intervention for valve repair or valve replacement if
symptoms persist
Secondary prevention
• An injection of 0.6-1.2 million units of benzathine penicillin G
intramuscularly every 4 weeks is the recommended regimen (every 3
weeks in endemic areas)
• Or can use oral therapy with penicillin V
• In case of allergy, macrolides or clindamycin can be used
prognosis
• Variables that correlate with severity of valve disease include:
▪ number of previous attacks
▪ length of time between the onset of disease and start of therapy
▪ sex (females is worse than males).
• Insufficiency from acute rheumatic valve disease resolves in 70-80%
of patients if they adhere to antibiotic prophylaxis.
• 80% of manifestation resolve within 3 months and the remaining
within 15 weeks.
Risk of recurrence
• Highest risk of recurrence within 5 years of the initial episode
• Greater risk with younger age at the time of the initial episode

• Generally, recurrent attacks like the initial attack (however, risk of

carditis and severity of valve damage increase with each attack)
Thank you…
Any Questions…