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Rheumatic fever
1.
2. An 11 year old Polynesian male presents with fever up to 39
degrees, joint pain and swelling, along with shortness of breath and
fatigue. The fever comes and goes at random times of the day. The
symptoms have been present now for 4 days. Two days ago, his right
knee was painful, tender and swollen, but today it has improved. The
shortness of breath occurs with walking. He also has some shortness
of breath with lying down flat when he is trying to sleep.
3. VS T 38.2,
HR 160,
RR 32,
BP 100/60,
oxygen saturation 94% in room air
160
94
100/60
32 38.2
4. HEENT: Enlarged, erythematosus tonsils with
exudates. Lungs are clear but with tachypnea.
Heart sounds are tachycardic with a holosystolic
murmur 3/6 heard at apex with radiation to axilla.
Audible apical S3 sound.
His left knee is swollen and extremely tender with
warmth. He has difficulty with range of motion but
can flex his knee 30 degrees passively. His right
ankle is very swollen and warm. He is unable to
walk due to pain.
5. ESR – 100 (<20 for young female)
CRP of 9.5 (normal 0-3)
ASO titre > 200 Todd units (peak value attainedweeks then
comes down to normal by 6 weeks)
Anti-DNAse B test
Throat culture: GABHStreptococciat 3
Group A streptococcus / streptococcus pyogenes / B-hemolytic streptococcus
6. Clinical course
Chest X-ray with cardiomegaly,
pulmonary congestion.
EKG reveals a prolonged PR interval
echocardiogram confirms severe mitral
insufficiency/mitral regurgitation
Enlarged liver
normal
abnormal
• left atrial hypertrophy;
• left ventricular
hypertrophy
• carditis
inflammation of the
myocardium that
caused delay in the AV
conduction,
7.
8. What is the main difference between Rheumatic Heart Disease
(RHD) and Acute Rheumatic Fever (ARF)?
a. In ARF there is an elevated ESR
b. In RHD there is a prolonged P-R interval
c. In ARF there is a history of arthralgias
d. In RHD there is evidence of chronic heart disease
e. In ARF there is evidence of erythema marginatum
the patient had severe carditis which caused his acute congestive heart failure, as manifestations of
ARF, but he subsequently develops chronic heart disease as a sequelae of the ARF carditis and thus it
would also be correct to describe him in terms of a more chronic form of the disease, namely
Rheumatic Heart disease (RHD)
10. • Mitral valve > Aortic valve
• The initial valvulitis of ARF results in valvular
insufficiency/mitral regurgitation
• if enough inflammation has occurred on the
valve leaflets of the mitral valve, the leaflets
may scar and become adherent to each other,
resulting in mitral stenosis - “fishmouth
stenosis”
• Infective endocarditis – microbial attachment
at rough fibrotic tissue
11.
12. • The acute arthritis of ARF will normally respond very dramatically to high dose
salicylate therapy
• aspirin
The treatment duration is usually 4 to 6 weeks or until the ESR or CRP returns to
normal. If it is stopped too early, the arthritis usually returns
• if there is evidence of severe carditis, then corticosteroids
• Antibiotic prophylaxis against streptococcal infections is utilized to prevent a
recurrence of ARF, and thus prevent further damage to the valves - intramuscular
benzathine penicillin, which is given every 4 week
Editor's Notes
In chreonic decompensated Mitral regurgitation – dilated/hypertrophied LA with excess blood – increase in pulmonary venous pressure – pulmonary edema.
tachypnea (due to temp) and tachycardia.
Murmur 3/6 – prominent but no loud.no thrill
Antistreptolysin O (ASO) titer is a blood test to measure antibodies against streptolysin O, a substance produced by group A streptococcus bacteria.
Anti-DNase B is an antibody produced by the immune system in response to a strep infection
ECG: first degree heart block
left atrial hypertrophy; left ventricular hypertrophy
The enlarged liver size gave further evidence of congestive heart failure. These findings are important to note, especially in a child with possible symptoms of orthopnea. Congestive heart failure is a severe form of carditis in ARF, and is managed more aggressively, often needing corticosteroids, diuretics, digoxin, and occasionally inotropic agents.
P-R interval is a measure of the atrioventricular (AV) conduction. Prolonged PR interval indicates AV block. Normal PR interval is 0.12 to 0.20 sec, slightly shorter in children with upper limit being 0.18 sec.•
In case of rheumatic fever, it is due to the inflammation of the myocardium that caused delay in the AV conduction, thus prolonged PR interval.
The polyarthritis must be migratory. This manifestation is one of the most common of the major criteria in ARF. Usually one joint becomes involved and over a few days resolves, then another joint(s) becomes involved as demonstrated in our case. Occasionally, the first joint does not resolve completely by the time the second joint becomes involved, and this is termed "additive arthritis", and also fulfills a diagnosis of migrating polyarthritis. In ARF, two or more joints are considered polyarthritis. If migrating polyarthritis is present you cannot use the minor criteria of "arthralgias", as virtually all the children with polyarthritis from ARF have a significant amount of pain. The most common joints involved are large joints, usually those that weight bear. Knees and ankles are most often involved, although elbows and wrists can also be involved. Metatarsophalangeal joints can be involved and one can screen for their involvement by squeezing them together, across the foot, and eliciting pain. The joint pain of ARF is typically very severe even if the visual findings are not very impressive. Merely touching the joint often elicits severe pain. Lower extremity joint involvement renders these patients non-ambulatory
he terms of Acute Rheumatic Fever and Rheumatic Heart Disease are sometimes confused. Proper use of these terms requires some knowledge of the disease entities even though their pathogenesis and relation to streptococcal infection is nearly identical. ARF is usually used to describe the initial or acute onset of the disease. In our case, this being the first initial presentation of the disease, it would be correct to call this ARF. The case fulfills modified Jones criteria as will be discussed below. However, as time goes on it is found that this child has a persistence of the murmur. He also had severe carditis which caused his acute congestive heart failure, as manifestations of ARF, but he subsequently develops chronic heart disease as a sequelae of the ARF carditis and thus it would also be correct to describe him in terms of a more chronic form of the disease, namely Rheumatic Heart disease (RHD). This term implies there has been significant valvulitis, enough to cause valvular scarring. This child is at an increased risk of requiring a valve replacement in the future, especially if he develops another episode of the disease, which puts great emphasis on him receiving long term penicillin prophylaxis, to prevent him from getting streptococcal disease and possible reoccurrence of ARF with worsening RHD.
More often, the carditis of ARF is not quite this severe, but can be problematic. The most common valve involved is the mitral valve. The second most common valve involved is the aortic valve. Classic mitral insufficiency sounds like a holosystolic murmur heard at the apex which radiates to the axilla. There are very few cardiac lesions that can be heard in the axilla. The murmur of aortic insufficiency is a diastolic murmur (difficult to hear) that is usually heard best at the upper left sternal border. There is often a decrescendo component to this murmur that is sometimes very high pitched. One should also listen for a rub which would indicate pericarditis and a gallop for evidence of congestive heart failure.
The initial valvulitis of ARF results in valvular insufficiency. Subsequently as RHD develops, if enough inflammation has occurred on the valve leaflets of the mitral valve, the leaflets may scar and become adherent to each other, resulting in mitral stenosis (usually seen late in the patient's course, sometimes after repeated episodes of ARF). The murmur of mitral stenosis is a diastolic murmur, although it is described as occurring in mid-diastole, rather then later in diastole like aortic insufficiency. Similarly, aortic stenosis may subsequently result from initial aortic insufficiency.
The acute arthritis of ARF will normally respond very dramatically to high dose salicylate therapy. The aspirin dose is 70-100 mg/kg/day divided into QID dosing with a maximum dose of 975 mg QID. Aspirin tablets come in 81 mg, 325 mg, and 975 mg. Use enteric coated tablets if available, and ask patients to eat prior to taking the aspirin. Monitor salicylate levels and liver function tests while on aspirin. Be very careful with ARF patients who have some elevation in liver function tests prior to being put on aspirin, since a low grade inflammatory hepatitis can be seen in ARF. The aspirin could aggravate this problem. The treatment duration is usually 4 to 6 weeks or until the ESR or CRP returns to normal. If it is stopped too early, the arthritis usually returns.
If the carditis is mild and the child is asymptomatic from a cardiovascular standpoint, then salicylate therapy is usually given. However, if there is evidence of severe carditis, then corticosteroids are indicated. Severe carditis is manifested by evidence of congestive heart failure (e.g., gallop rhythm, cardiomegaly, etc.) or severe myocardial disease (e.g., two valve disease or a new or a worsening arrhythmia). Close follow-up and evaluation by the cardiology service is warranted. Repeat echocardiograms will be needed. Corticosteroids are indicated for severe carditis under the direction of a cardiologist. Prednisone is usually given for 2 to 3 weeks followed by aspirin while the corticosteroids are tapered.
Antibiotic prophylaxis against streptococcal infections is utilized to prevent a recurrence of ARF, and thus prevent further damage to the valves. Long term prophylaxis needs to be carefully described to the parent and child. Many of the families do not understand why the child needs penicillin injections when he or she feels fine, following the episode of ARF. Many mistakenly think the injections are for the arthritis and therefore do not comply with this regiment once the arthritis has resolved.
There is currently some debate about whether the penicillin injections should be given every 3 or 4 weeks, as well as, the length of treatment (10
