ACUTE RHEUMATIC FEVER
Acute rheumatic fever is defined as a systemic illness characterized by
fever polyarthritis and carditis. The importance of the disease lies in the fact that
after an interval of several years damage to the heart valves may become
manifest in the patient leading to chronic disability and even fatal outcome arising
out of one of the complications. It is mainly an infection prevalent in the young
age. It is still very common in the developing countries where as it has been
controlled in developed countries.
It is caused by a delayed antigen antibody reaction to infection with group
A B hemolytic streptococcal infection of the throat. The patient develops the
throat infection first in the form of a pharyngitis or tonsillitis by streptococci, which
might subside within a week or so with or without treatment. These forms of
upper respiratory infections are very common in young children living in
circumstances of poor hygiene and over-crowding. But only a small proportion of
these children develop acute rheumatic fever 2-4 weeks afterwards. It is
important to note that similar infection caused to the skin in the form of infected
scabies or pyoderma often leads to acute glomerulonephritis and not acute
The child usually presents with sudden onset of high grade and
continuous fever and painful swelling of one or more major joints of the body.
The usual joints that are affected are the elbow, wrist, knee and ankle. The
involvement is symmetrical and usually spares the smaller joints unlike in
rheumatoid arthritis, which is the immediate differential diagnosis.
The characteristic pattern of involvement is called flitting or migratory. This
means that as one joint involvement is decreasing another joint gets affected. At
any point of time two or three joint will be affected. This is also unlike in
rheumatoid arthritis where the involvement is uniformly symmetrical and
simultaneous. There is painful restriction of mobility and the patient may remain
bedridden for weeks together.
After one or two weeks the patient may develop another major
manifestation termed as carditis. This is a pancarditis meaning inflammation of all
the layers of the heart namely the pericardium, myocardium and the
endocardium. The patient will then complain of chest pain, palpitation and
dyspnoea along with fever. Some patient may develop pitting pedal edema of
both legs due to development of cardiac failure
The next major manifestation is rheumatic chorea, otherwise called as
Sydenham’s chorea. This is usually seen in young children with rheumatic fever.
It manifest in the form of repeated but unintentional movements of the upper
limbs producing bizarre jerky movements, which are also done often without any
purpose. The child also is irritable and restless and fidgety and always moving
his torso except during sleep. This form of involuntary movements are very
important because the chance of developing a carditis soon and valvular heart
disease later is much more in these patients than in rest of the patients without
The fourth major manifestation of acute rheumatic fever is erythema
marginatum. These are usually seen only in fair skinned people. It is in the form
of reddish macular lesions with raised edges and blanching center. They may
later coalesce and become bigger. They are mainly seen over the ankle joints
and over the shin.
The fifth major manifestation is sub-cutaneous nodules. These are seen
mainly over the extensor aspects of the limbs and over the tendons. These are
small round firm and non-tender nodules. They are smaller than the nodules
seen in rheumatoid arthritis.
The minor clinical manifestations of importance are fever, polyarthralgia in
the absence of polyarthritis and the previous history of streptococcal infection of
The child is usually sick, febrile and bed-ridden. There will be fever of high
grade. Tachycardia, which is sometimes out of proportion to the fever and
attributed to the carditis, is usually prominent. Normally the heart rate increases
by eighteen beats per minute for every degree rise in Celsius temperature. Here
it may be much more than that. Blood pressure is maintained normally in most of
the cases and the jugular venous pressure may also be normal, unless there is
The heart size is usually enlarged as evidenced by a downward and
outward displacement of the apex. The appearance of a new murmur or the
change in characteristics of an already existing murmur is very suggestive of
rheumatic valvulitis. The usual murmurs are those due to mitral regurgitation and
aortic regurgitation. The former is a pan-systolic murmur and the latter an early
diastolic murmur. Some times there can be amid-diastolic rumble heard due to
valvulitis of the mitral valve producing obstruction to blood flow across the mitral
valve. This murmur, which might disappear later is called Carey Coombs murmur
The investigations to be done in a suspected case of acute rheumatic
fever are important because the diagnosis is also based on them.
These are the routine blood examination, which may reveal leucocytosis,
raised erythrocyte sedimentation rate and an increase in the C reactive protein
levels all of which indicate acute inflammation.
The electrocardiogram will reveal tachycardia, evidence of cardiac
chamber enlargement and evidence of first degree hear block, which manifests
as a prolongation of the PR interval or second-degree heart block, which
manifests as periodically dropped beats.
The X-Ray of chest will also show features of cardiomegaly and
pulmonary venous congestion.
The diagnosis of acute rheumatic fever is made depending on a mixture of
clinical signs and investigations. This is now based on the revised Jones criteria.
Two or more major manifestation or one major and two or more minor
manifestations are essential for the diagnosis. The minor manifestations included
in the list among investigations are leucocytosis, high ESR, increased C Reactive
Protein and the presence of first degree or second degree heart block in the
electrocardiogram, In addition to these an evidence of previous streptococcal
infection of the throat in the form of raised ASO titre or positive throat swab
culture must be there to arrive at a diagnosis.
The child is given complete bed rest during the acute phase of rheumatic
fever. This is important in not only preventing relapses, but also in reducing the
incidence of carditis and permanent valvular damage. The bed rest must be
continued for 2 to 4 weeks only after the clinical and investigation parameters like
the ESR and C reactive protein have fallen to normal ranges.
Children and young adults, when given in large doses best tolerate acetyl
salicylic acid in enteric-coated forms. Two to three tablets of 325mg are given 4
to 5 times a day after food and along with antacids and H2 receptor blockers like
ranitidine in order to minimize the gastric irritation. This dose is continued till the
ESR falls to normal and then slowly tapered off.
Corticosteroids are indicated in presence of severe arthritis and carditis.
Prednisolone is the drug of choice and it is given in the dose of 1mg per kg body
weight per day in two or three divided doses along with food.
Treatment to eradicate infection of the throat with streptococci is
important. This can be achieved by a course of crystalline penicillin 10lakh units
given IM or IV 6 hrly for 7 days followed by oral administration of phenoxymethyl
penicillin 500mg twice daily for ten days. There after for prevention of recurrence
it is advised to continue the oral penicillin on a daily basis till the patient reaches
the age of twenty-five years or in case of carditis thirty years or at least five years
after the last attack of acute rheumatic fever