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Rheumatic Fever

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Khalid Massalha

on 1 May 2011

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Transcript of Rheumatic Fever

Introduction Facts.. GOOD! BAD! Developed Countries Developing Countries 100-200 0.5 Incidence (per 100,000 child per year 12 Millions affected
40,000 Deaths annually Risk Factors Agent Host Environment group A beta hemolytic streptococcus Must be Pharyngeal
school-age children
silent in 1/2 of the patients Virulence-dependent! M-Protein


lipoteichoic acid
hyaluronate capsules 1) resist phagocytosis
2) Adherence
3) >100 types, few are virulent
6-15 years
both sexes equally affected
All ethnic groups

Genetics higher predisposition to RF in certain families

higher incidence of concordance of RHD among monozygotic twins (18.7%) compared to dizygotic twins (2.5%)

specific B-cell alloantigen (D8/17)

Overcrowding
poor access to health care
peak in late winter and early spring
"Hot-Spots": Sub-saharan Africa, Pacific Nations, Australasia, the Indian subcontinent Pathogenesis latent period molecular mimicry Cross Reaction Autoimmunity Humoral Cellular M-Protein with human tissues cross-react Valves myocardium cartilage nervous system changes in the number and function of lymphocytes
higher production of cytokines Clinical Manifestation latent period from 1 to 3 weeks after streptococcal pharyngitis
Acute or Insidous Arthritis commonest mode of onset: 60 to 75% as a major sign
migratory polyarthritis Pain > Swelling
Large > Small
Knees: 75%
ankles: 50% Self-limited, lasts days - 1 week.
response to Aspirin or other NSAID’s in 24 hours is considered an important clue to the diagnosis. prolonged duration as poststreptococcal reactive arthritis
Monoarthritis: related usually to early treatment with NSAIDs Carditis 40-50%
Most serious! RHD or Fatal!
PANcarditis: endocardium, myocardium and pericardium
mitral regurgitation in 98% !
Asymptomatic severe Physical Examination Tachycardia mitral regurgitation apical
high pitched
blowing
holosystolic murmur not related to fever the severity of Prognostic Significance mild-mod disappears on follow-up Aortic regurgitation 20% Pericarditis- small-mod effusion
6 to 15%
suspected with precordial discomfort/friction rub

Myocarditis- No significant myocyte damage Severe carditis- Cardiac failure Cardiomegaly Aortic valve showing active valvulitis. The valve is slightly thickened and displays small vegetations – "verrucae" Stenotic mitral valve seen from left atrium, showing fusion of commissures, thickening and calcification of the cusps Chorea Sydenham’s chorea
Chorea Saint Viti involuntary movements
diffuse hypotonia
dysarthria
psycologic dysfunction
Emotional lability
OCD

1/3 of patients
Isolated: latency to 9 months
No evedince of GAS infection
acute attack: along with Carditis
recurrence rate: 32%

learns to walk again after suddenly being bed-ridden for six weeks with Sydenhams Chorea, aka St Vitus Dance in January 2009, at aged 6. The illness lasted 12 weeks, getting progressively worse for the first six weeks. For eight weeks she was disabled, unable to control movement in her limbs or face, and speech was unintelligible. We looked after her at home with excellent support from paediatric neurological Consultants at Evelina Children's Hospital and Medway Hospital. Penicillin and sodium valporate were administered. She returned to school 12 weeks after the first signs of the illness became noticeable, in January 2009. The co-ordination of her eyes for reading recovered two months after going back to school, and her handwriting recovered gradually durng the next 12 months. She has now made a complete recovery. There is a one-in-five chance of Sydenhams recurring within two years. Subcutaneous nodules Subcutaneous nodules rarely seen (<10%), associated with severe carditis!
after few weeks of Cardiac findings


small (few mm to 1-2 cm in size), round, firm, painless, multiple on bony prominences or extensor tendons without signs of skin inflammation Erythema Marginatum <5%
nearly always indicative of underlying carditis
trunk and proximal limbs non-pruritic, transient rash, 1-3 cm in size with a slightly raised periphery and clear central skin. Erythema Marginatum Others Arthralgia

Fever: in all patients with arthritis, never in isolated chorea. +/- 1 week

Meningitis,
Encephalitis.
Pleurisy,
Pneumonitis.
Vasculitis: e.g. mesenteric vasculitis, with acute abdomen Laboratory recent streptococcal infection systemic inflammation heart disease Throat cultures Rapid antigen detection Streptococcal antibody tests Only 20% is Positive for GABHS in patients with RF ..as a result of the latend period High specificity (>95%)
low sensitivity.
the most common test for strep A.

detecting the presence of a carbohydrate antigen unique to group A Strep.
Gargling, eating or other infusion of liquids affects the results anti-streptolysin O (ASO)

anti-DNaseB
anti-hialuronidase after 7-10 of infection
peak from: 2wks - 6mos
mostly, no evedince with Chorea (long latency)
20% false negative All 3 tests together:
95% sensitivity Acute phase reactants non-specific, highly sensitive
helps to confirm and monitor
reflects the magnitude Erythrocyte sedimentation rate (ESR) C-reactive protein > increased PR interval
present in 28-40% what's wrong?! Chest radiograph of an 8 year old patient with acute carditis before treatment Same patient after 4 weeks 1) ECG 2) Chest radiograph 3) Doppler Echocardiogram chamber size
systolic ventricular function
pericardial effusion
valve lesions and abnormal regurgitation
able to identify subclinical carditis Diagnosis Modified Jones' Criteria Majors Carditis
Arthritis
Chorea
subcutaneous nodules
erythema marginatum Minors fever
CRP or ESR
arthralgia
Prolonged PR Diagnostic:
Evidence of Strep A infection
PLUS
Two Majors
One major + two minors
Three Exceptions: 1) recurrent attacks
2) insidious or late onset carditis
3) chorea as the only manifestation of RF Treatment Primary Prevention Secondary Prevention Supportive eradication of GAS from the pharynx Benzathine Penicillin (G)
Patients > 27 Kg: 600.000 U, IM
Patients < 27 Kg: 1.200.000 U, IM

Penicillin V
Children: 250mg 2-3 times daily, PO (10 d)
Adolescents: 500mg 2-3 times daily, PO (10 d) Penicillin-allergic Erythromycin 20 mg to 40 mg/kg in 2 to 4 divided doses for 10 days prevent recurrent attacks of acute RF Benzathine penicillin G
1,200,000 U every 4 weeks*, IM

Penicillin V
250mg twice daily, PO

Sulfadiazine
< 27 Kg: 0,5 g/day
> 27 Kg: 1 g /day

Erythromycin
250mg twice daily, PO risk factors of recurrent rheumatic fever
The number of previous attacks
Time since the last attack
Risk of exposure to streptococcal infections
Patient age
Presence or absence of cardiac involvement Even with optimal adherence, the risk of recurrence is higher in individuals receiving oral prophylaxis throat cultures for household contacts full treatment for positive, even if assymptomatic Carditis Conventional therapy for heart failure
valve surgery: Repair or treatment
Aspirin 80 to 100 mg/kg per day in children and 4 to 8 g/day in adults relief of symptoms No reduction in the risk of heart valve lesions was observed with corticosteroids or intravenous immunoglobulin Steroids 2 mg/kg / day – maximum 60 mg/day high dose for 3wks, then decrease 20% every week Arthritis & Rash Aspirin 80 to 100 mg/kg per day in children and 4 to 8 g/day in adults continued until
all symptoms have resolved or
Normalization of inflammatory markers (CRP, ESR) antihistamines helps alleviate pruritis Outcome chorea or arthritis have the best prognosis
HOWEVER, RHD can be diagnosed years after the initial attack of chorea! Carditis Total recovery death due to cardiac failure One Valve?
58-74% full recovery multiple valve?
Cardiomegaly
Heart failure heart spared in 1st attack, likely to be spared in subsequent occurrences
Chances of recurrence are higher (50%) within the first 6 months of the initial attack and lessen to only 10% after 5 years Mitral > Aortic persistent valvular damage occurs in 50% that manifest with murmur! Do Rates of Arthritis and Chorea Predict the Incidence of Acute Rheumatic Fever?

Robert D. Tunks, M.D.
Mario A. Rojas, M.D., M.P.H.
Kathryn M. Edwards, M.D.
Michael R. Liske, M.D.

Monroe Carell Jr. Children’s Hospital at Vanderbilt

Vanderbilt University Medical Center
Nashville, Tennessee

Pediatrics International accurate ARF incidence figures in Africa, a continent felt to be particularly affected by ARF, remain largely unknown
One potential impediment to determining the incidence of ARF in developing countries is the difficulty in recognizing this multi-system disease in settings of limited medical resource We hypothesized that the more easily recognized, non-cardiac
features of ARF, namely joint involvement and chorea, might serve as the primary index findings Methods retrospective review of inpatient and outpatient hospital billing records from 1998 to 2008 from Vanderbilt Children’s Hospital.

less than or equal to 18 years of age who met the Jones criteria for the diagnosis of acute rheumatic fever were included

A literature search was also conducted using a variety of internet resources such as PubMed and SUMSearch
Studies were selected for inclusion if
data were presented in a format that allowed the enumeration of the specific manifestations of acute rheumatic fever.
published in the English language.
used the Jones criteria for the diagnosis of ARF
enrolled more than 40 patients.
RESULTS 59 cases
91% presented with joint manifestations or chorea
80% satisfied the Jones criteria for ARF
Conclusions Most patients presenting with ARF have either joint symptoms or chorea, features that could be recognized by community health workers and individuals with limited medical training. The referral of patients presenting with these manifestations for further evaluation might improve detection rates of ARF in resource limited countries and lead to improved estimates of disease burden Case Report Ethan, a 4-year-old boy who had always been in good health, complained one morning that he was unable to stand up. He had a fever and his knees were swollen and painful.

two of his siblings had had ‘strep throats’ 2 months ago which had been treated with amoxicillin

X-ray of his knees showed no pathology
ESR = 98 mm/h

No improvement within a week, a murmur has been detected. History Physical Examination Ethan weighed 16.8 kg (normal) and was 107 cm tall (normal)
HR=107 (rapid) , RR=22 (normal) , BP=100/47
systolic murmur heard best at the apex of the heart.

Echo: incompetence of his mitral and aortic heart valves and a dilated left atrium and ventricle.
Lab WBC= 12,600 , PLTs= 440,000 (Both elevated)
anti-streptolysin O = 1:1600, Positive
anti-strep DNase B titer = 1:1920, positive Case Report Secondary Prevention Rheumatic Fever Khalid Massalha
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