REVIEW FOR THE PRIMARY CARE
PHYSICIAN
RHEUMATIC FEVER
Sheila Knupp Feitosa de Oliveira and Marcia Ribeiro
Keywords: arthritis, chorea, rash, nodules
Contact:
Sheila Knupp
Pediatric Rheumatology
Universidade Federal do
Av. Sernambetiba 2940, Bl.F202
Rio de Janeiro, Brazil 22620-172
e-mail: sheilaknupp@terra.com.br
INTRODUCTION
In
spite of the dramatic decline of acute rheumatic fever (RF) and rheumatic heart
disease (RHD) in developed nations, there is still a significant morbidity and
mortality in developing countries. Although accurate data on the
incidence of RF are lacking in developing countries, estimates range from 100
to 200 per 100,000 school aged children per year while in developed nations the
mean annual incidence is 0.5/100,000 in children of the same age (1). Over 12
million people are affected by rheumatic fever worldwide and approximately
40,000 deaths result from rheumatic heart disease annually, mainly among
children and young adults. It is estimated that two million patients now
require heart surgery due to RF and one million more will require heart surgery
in the next 5 to 20 years (2).
EPIDEMIOLOGY
Risk factors pertaining to the 3 components of
classical epidemiologic triad – agent, host and environment – have been
identified as important determinants of disease distribution in the population.
The etiologic agent – To
initiate acute RF, the site of infection caused by a strain of group A beta
hemolytic streptococcus (GABHS) must be pharyngeal. Yet, not all strains that infect the pharynx
cause acute RF. All age groups can be infected by streptococcus, but RF usually
occurs among school-age children, where the prevalence of group A streptococci
isolated from sporadic pharyngitis varies from 20 to 35%. The infection is
asymptomatic in half of the patients and the diagnosis can be documented only
retrospectively by a rise of antistreptococcal antibodies. It is important to differentiate the carrier
state from actual infection. Detection
of GABHS from the throat does not necessarily indicate a recent active
infection and a rise of an immune response to the bacteria is an additional
prerequisite (3).
Attack rates of RF are related to the virulence of the
infecting strains and possibly by the capacity of the immune response of the
host. In the 1950’s, it was reported that 3% of untreated epidemic
streptococcal pharyngitis in military recruits in the
Epidemiologic observations during epidemics of
streptococcal infections associated with RF outbreaks suggest that the
“rheumatogenic” potential could be closely linked to the streptococcal
virulence. The virulence appears to be linked to the protein M present in the
streptococcal cell wall. The protein M has the ability to resist phagocytosis
by neutrophils (13, 14) and may also be an attachment factor providing the
bacterium with an adherence advantage (15). Although there are approximately
one hundred different serotypes of streptococcus based on the protein M, only a
limited number of streptococcal M-types have been isolated or have been
associated with one or more outbreaks of RF.
Other extracellular and cell-associated products of
streptococcus could play a role in the virulence. These products include the
presence of hyaluronate capsules that may make strains more resistant to phagocytosis
and lipoteichoic acid on the streptococcal surface that is responsible for the
ability to adhere to pharyngeal mucosa.
The
host - RF affects
mainly children between 6 and 15 years, is uncommon in children under 5 years,
and rare in children younger than 3 years. The frequency of the disease
declines after puberty and is rare in adulthood. Both sexes are equally
affected although there is a slight predominance of chorea in females. RF is
reported in all ethnic groups although living conditions, socioeconomic status,
and access to health care may be more important and represent potential
confounders.
The first evidences of genetic predisposition for the
disease were based on a higher predisposition to RF in certain families (16,17)
as well as the higher incidence of concordance of RHD among monozygotic twins
(18.7% of 56 twin pairs) compared to dizygotic twins (2.5% of 40 pairs) (18).
More recently a variety of genetic markers such as class II HLA haplotypes and
a specific B-cell alloantigen (D8/17) were noted to be associated with a higher
incidence of RF in certain populations. In fact, significant associations were
encountered for the class II HLA-DR alleles, but these differences were related
to the ethnicity of the populations studied. The diversity of these
associations raised a number of questions regarding the absence of a clear
association between susceptibility to RF and a specific HLA-DR (19-22). The
specific B-cell alloantigen (D8/17) has been identified in a high percentage of
B cells in most RF patients, but a commercial test for this alloantigen is not
yet available to the clinician.
Furthermore, the costs of these tests and the low specificity and
sensitivity do not allow their use as a preventive strategy directed towards
individuals predisposed to RF (23).
The environment – Overcrowding and poor access to health care, both linked to
socioeconomic development, seem to be the most important determinants of
disease distribution. Seasonal variations in RF are not pronounced in the
tropics (24), although in temperate climates, streptococcal infections have a peak in late winter and early spring.
RF
is a multisystemic disease affecting multiple organs: heart, joints, central
nervous system and skin (1). The beginning of the symptoms usually occurs after a latent period from 1 to 3
weeks (mean 18 days) after a streptococcal pharyngitis caused by GABHS and does
not become shorter in repeated attacks. Onset may be acute or insidious. Usually acute attacks are associated with
arthritis or carditis with pericardial effusion. Insidious onset of RF is seen
in some cases of carditis and chorea, in which early behaviour changes may be
misinterpreted.
Arthritis- Arthritis
is the commonest mode of onset and tends to occur early in the disease. In the
first attack of RF, approximately 60 to 75% of children have arthritis as the
major sign.
The classic pattern is migratory polyarthritis with
overlap of joint involvement. Pain is more marked than swelling and frequently
leads to functional impairment. In general, arthritis starts in large joints
but it can also affect the small ones. The most common sites of involvement are
knees (75%) and ankles (50%), followed by elbows, wrists, hips and small joints
of feet (12-15%). Shoulders and small joints of the hands are the least
involved (7-8%). Rarely, spine and temporomandibular joints are involved.
Arthritis is usually self-limited: each joint swelling lasts from few days to
one week and the total episode rarely lasts more than one month. The
therapeutic response to anti-inflammatory doses of aspirin or other NSAID’s is
considered an important clue to the diagnosis as it stops pain in 24 hours and
the other inflammatory signs in 2 or 3 days.
Some patients can exhibit a different pattern of
arthritis adding some difficulty to the diagnosis, mainly when there is no
other major sign of RF. They can show an additive and more prolonged duration
of arthritis and a weak response to NSAID’s, in the same way as it has been
described in poststreptococcal reactive arthritis (34-35). Monoarthritis can
occur but it is usually
related
to the early use of nonsteroidal anti-inflammatory drugs before the disease is
fully expressed in its migratory pattern.
Carditis - Heart involvement occurs in
40-50% cases of initial attack. It is the most serious manifestation as it can cause a permanent damage - rheumatic heart disease
(RHD) - or can be fatal.
All
cardiac structures such as endocardium, myocardium and pericardium may be
involved but the lesion that defines carditis is mitral regurgitation in 98%
(being the only involved valve in 70 to 75% of the patients) or isolated aortic
regurgitation in 2%.
Carditis onset is quite
variable. Its severity may range from asymptomatic to severe. It can
be diagnosed as an isolated manifestation or together with other major signs. Asymptomatic
carditis often is detected in patients who present with arthritis or develop
chorea. In general, older patients present more with
carditis associated with arthritis.
The articular pain often brings the patient to the doctor who then makes the
diagnosis of carditis as well. In these cases, usually carditis appears in the
first 2 weeks of arthritis onset. After the first two weeks, the chance of
developing carditis is reduced. However, younger patients often have an insidious onset of isolated carditis, and 50%
of all patients diagnosed with RHD don’t recall any past history of joint pain.
Auscultatory
findings are the most indicative findings of carditis. Tachycardia and murmur
are the commonest clinical manifestations of carditis. Tachycardia is not
related to fever and basal pulse rate is high. Mitral regurgitation is manifested by an
apical, high pitched, blowing, holosystolic murmur. It may be accompanied by a
low-pitched, short, mid-diastolic murmur (Carey-Coomb’s murmur) which does not
have a presystolic accentuation typical of mitral stenosis and disappears on
follow-up. The severity of mitral regurgitation is of prognostic significance,
as in the majority of patients with mild
or moderate mitral regurgitation, the lesion disappears on follow-up (36).
Aortic regurgitation is present in 20 to
25% of the patients and is manifested by a high-pitched, soft, decrescendo
murmur. Tricuspid valve and pulmonary valve lesions are rarely clinically
significant (37). If the valvular insufficiency persists or later on evolves to stenosis,
rheumatic heart disease (RHD) is the accepted terminology.
Pericarditis
is associated with small to moderate effusions but never produces cardiac
tamponade. Clinically it is present in 6 to 15% of the patients and is usually
suspected in the presence of precordial discomfort or pain or a pericardial rub
(scratchy, leathery sound altered by varying pressure of stethoscope) heard in
both phases of cardiac cycle. Myocarditis is confined to the interstitium and
does not usually result in significant myocyte damage, consistent with the
preserved left ventricular ejection phase indices seen in patients with active
rheumatic activity.
Severe carditis comes with
cardiac failure and may be fatal in the acute stage. In cases of severe carditis,
cardiomegaly, a third heart sound and symptoms of cardiac failure may be
present. It is now believed that
rheumatic myocarditis is not the primary cause congestive heart failure in patients with acute carditis. It is important
to note that pathologic data show little damage in the myocardium in patients
dying from RF (38) and cardiac failure does not occur in the absence of
significant valvular lesions (39).
The diagnosis of a new flare
of carditis in a patient with an established RHD may be difficult. Cardiac manifestation such as pericardial
friction rub, a new murmur or worsening of a pre-existent one, increasing heart
size, unexplained congestive cardiac failure in the presence of evidence of
recent streptococcal infection should be considered highly suspicious for the
diagnosis of recurrence of active rheumatic carditis.
Silent carditis is a term
used to define patients with subclinic carditis, without murmur but in whom
valve regurgitation is detected by Doppler echocardiography. However, according to the last revision of
Jones’ criteria in 1992, this exam by itself can not be considered as evidence
of carditis. The use of echocardiography
to document carditis without auscultatory findings remains controversial.
Chorea - Chorea occurs in approximately one-third of the patients
with RF. It is characterized by an array of neuropsychiatric symptoms that vary
in severity, timing and character (40). The latent period can be long, as much
as 9 months, so that no evidence of previous streptococcal infection can be
found. The onset can be associated to other RF manifestations, mainly carditis,
during an acute episode or as an isolated form characterizing the “pure” chorea
in 35%. Follow up of patients with pure rheumatic chorea has shown
RHD in 23 % in a 20-year period (41). Thus, chorea may be a good marker for
future occurrence of valvar heart disease.
Clinically, the main features are involuntary
movements, diffuse hypotonia, dysartria, emotional disorders and less
frequently by other neuropsychiatric manifestations (42). Emotional lability precedes the onset of chorea
movements. Most reported are impatience, irritability and inattention to
school-work. However, in the last
decade, more severe neuropsychiatric abnormalities, such as obsessive
compulsive and tics disorders, have been associated with chorea. Incoordination
and involuntary movements are initially perceived as clumsiness and as a
tendency to drop objects. Then, purposeless, unilateral (hemichorea) or
bilateral movements become evident. All
voluntary muscles may be involved although they can be suppressed voluntarily
for short time. During the physical examination, the
physician should observe involuntary movements in face, tongue, hands, where
they are more evident. Some tests to detect them include: quality of handwriting, slurred speech when counting from 1 to
ten, milking sign when gripping the examiner hand, spooning or dishing of hands
(flexion of wrist and hyperextension of MCP) when extending hands and pronator
sign (pronation of hand) when raising hands above the head. The difficulties
can become more evident when asking to perform 2 or more motor functions, one
after another.
Subcutaneous
Nodules - They are infrequent as they occur in less than 10% of the patients and
their presence usually suggests an underlying carditis. They are rare in
adults. Usually they appear after some weeks after the first few weeks of
cardiac findings. Subcutaneous nodules are small (few mm to 1-2 cm in size),
round, firm, painless, multiple on bony prominences or extensor tendons without
signs of skin inflammation. They are better felt than seen.
Erythema
Marginatum -
This is an infrequent manifestation of RF (less than 5%) and due to its
evanescent nature and lack of associated symptoms, it can often be missed in
patients with dark skin. It is nearly always indicative of underlying carditis.
It is an early manifestation of RF but may reappear at later stages. It is seen
on the trunk and proximal limbs, but the face is spared. It is a non-pruritic,
transient rash, 1-3 cm in size with a slightly raised periphery and clear
central skin.
Other
manifestations:
Arthralgia
and fever are not rare but are not specific of RF, being considered minor signs
according to Jones’ criteria. Arthralgia can be present for days or weeks and
should be considered as a minor sign only in patients without arthritis. Fever
usually occurs in all patients with arthritis, can be low in carditis and never
occurs in isolated chorea. There is no characteristic pattern and often lasts
only 1 week.
LABORATORY INVESTIGATIONS
There
is no specific diagnostic laboratory test for RF. Lab exams can only
demonstrate a recent streptococcal infection, the presence of inflammation, and
the presence and severity of heart disease. Other tests can be performed
initially to exclude other diseases in the differential diagnosis.
Rapid antigen detection – The
specificity of this test is high (over 95%) but not the sensitivity (43). Its positivity is equivalent to a positive
culture throat.
Streptococcal antibody tests – GABHS have
many antigens and antibodies directed towards some of them have been utilized
to identify a previous streptococcal infection even in asymptomatic cases. The
most utilized is anti-streptolysin O (ASO). When the first
symptoms of RF present, titers generally are high due to the latency period. ASO antibodies start to appear 7 to 10 days
after onset of the infection, reach the maximum between the second and third
weeks, and maintain this plateau for 3 to 6 months and then decline
(44). As chorea has a long latency period, many times there is no evidence of
streptococcal infection at the time of diagnosis. If a test is positive in a patient with arthritis, it does not mean that
the diagnosis is RF but only that the patient had a previous streptococcus
infection.
Unfortunately, in
approximately 20% of patients with RF, the ASO titer may be normal. In these
cases, this test should be repeated after 2 or 4 weeks to check if the titer
increases. If it continues to be negative, other antibodies, such as anti-DNaseB
and anti-hialuronidase, should be performed in order to improve the capacity to
confirm a previous infection. All three tests together establish the diagnosis
of previous streptococcal infection in 95% of the patients (45). However, in
most of developing countries, where the incidence of RF is high and resources
are limited, the only test available is ASO and sometimes it seems better to
treat a suspected case of RF despite a normal ASO titer result.
Acute phase reactants are
non-specific and only indicate the presence of an inflammatory process. They
help to confirm and monitor the acute phase of the inflammation. Erythrocyte
sedimentation rate (ESR) and C-reactive protein are the main tests used in RF
as both are often abnormal during the period of carditis and arthritis. Neither
is specific but both are very sensitive and reflect the magnitude of the
inflammatory process (rheumatic activity). C-reactive protein is better than
ESR because it is should be negative in healthy subjects whereas false positive elevations of ESR may occur.
Tests for recent carditis:
Electrocardiographic abnormalities can occur during
an episode of acute rheumatic carditis. The most important finding, although
non-specific, is the increased PR interval, present in 28-40% cases with
carditis.
Currently, chest radiographs are not important in
the diagnosis of rheumatic fever as Doppler echocardiography is the best
non-invasive method to evaluate and follow cardiac changes. It can evaluate
chamber size, systolic ventricular function, detect presence of pericardial
effusion and demonstrate absence or presence of valve lesions and abnormal
regurgitation. Doppler
echocardiography is able to identify subclinical carditis in patients with RF
who do not have audible murmurs.
PATHOLOGY
Arthritis - Histologically, there is swelling of the articular
and periarticular surfaces, but there is no erosion of the articular surface. Synovial fluid may be turbid but
non-purulent. Initially there is a predominance of neutrophils but later on,
mononuclear cells predominate. Fibrin may be enmeshed with the exudative cells.
Swelling and fibrinoid degeneration of the connective tissue occurs.
Carditis - The morphologic
hallmark of cardiac involvement in RF is the Aschoff body which is not
described in any other cardiovascular disorder. Typically it is a granulomatous
lesion, round to oval, usually less than 1 mm in size and found almost always
in the endocardium, subendocardium or perivascular regions of myocardial
interstitium. Aschoff bodies may be seen years after the initial illness and do
not correlate with the activity of the disease.
Usually
pericarditis with myocardial damage predominates in severe cases. Pericarditis
is accompanied by a serofibrinous effusion, but does not cause constriction at
any time. Myocardium is flabby, edematous or pale. Chambers are enlarged. Heart
enlarges due to dilatation of chambers or hypertrophy of the heart musculature.
Within the muscle there is an exudative inflammatory response with
lymphocytosis and plasma cells in the interstitium. Aschoff bodies are much
less frequent in the myocardium (5% of cases) compared to the endocardium (72%)
(39).
Endocarditis is always present. Acute
inflammation of valves is characterized by tiny translucent vegetations of 1 to
2 mm in diameter on the atrial surface at sites of the valve closure and on
chordae tendinae. The valves have inflammatory infiltrate, edema and
vascularization. Repeated fibrin deposits on the valve cusp results in fibrosis
of the valvular ring causing stenosis (e.g. mitral stenosis). Later,
commissures may be fused and chordae tendinae may be retracted and fused. Valve
lesions are often responsible for cardiac failure. Calcification of valve
leaflets occurs over a time. The most frequent valves involved are mitral and
aortic valves, while right sided valves are rarely involved. The endocardium
and subendocardium regions away from the valves are often inflamed and Aschoff
bodies are frequently present.
Chorea - The
main histological features were described in the cortex, cerebellum and basal
ganglia. They consist mainly in perivascular infiltration, petechial
hemorrhages, and hyalinisation of small blood vessels.
Subcutaneous Nodules - Histologically,
a nodule consists of fibrinoid material in strands with clear space in between.
There is much edema with very few cells- fibroblasts or histocytes and
occasional lymphocytes. There is no palisading characteristically seen in
rheumatoid nodules.
DIAGNOSIS
In spite of significant advances, there is no single
laboratory test to establish a diagnosis of RF.
It is still based on Jones’ criteria, proposed initially in 1944 and
reviewed and modified four times, in order to avoid missed diagnosis or
overdiagnosis. The most important items are the identification of at least one
major sign (polyarthritis, carditis, chorea, erythema marginatum and
subcutaneous nodules) and the detection of a previous streptococcal infection.
In the last revision of Jones’ criteria (1992) (46) (Table. 1), the basic rule for the
diagnosis - presence of two major or one major and two minor signs together
with an evidence of streptococcal infection -
was not altered but, for the first time, this formula was to apply only
to the initial attack of RF. They also recognized and accepted three exceptions
where diagnosis could be done without strict adherence to the criteria:
recurrent attacks in which signs and symptoms could be less apparent, insidious
or late onset carditis or chorea as the only manifestation of RF (pure chorea).
In these patients, the diagnosis should be presumptive until other causes have
been excluded.
Table
I: Modified Jones' Criteria for diagnosis of RF:
|
MAJOR MANIFESTATIONS |
MINOR MANIFESTATIONS |
|
Carditis |
Fever |
|
Polyarthritis |
Arthralgia |
|
Chorea |
Elevated
acute phase reactants: erythrocyte
sedimentation rate, C-reactive protein |
|
Erythema
marginatum |
Prolonged
PR interval on ECG |
|
Subcutaneous
nodules |
|
|
SUPPORTING
EVIDENCE OF ANTECEDENT GROUP A STREPTOCOCCAL INFECTION: Positive throat culture or
rapid streptococcal antigen test or an
elevated
or rising streptococcal antibody titer |
|
Treatment of RF should be directed toward
suppressing the ongoing inflammatory process in its different presentations,
eradicating GABHS still present in the throat, and preventing new recurrences
that could cause more damage in the future.
Treatment of the clinical
manifestations
Acetylsalicylic acid was the
first anti-inflammatory drug to be used in the treatment of RF. In arthritis,
anti-inflammatory doses usually are used as a diagnostic test as the response
is often dramatic. Pain ceases in one
day and the other inflammatory signs in two days, a pattern that is not
observed in several other causes of polyarthritis. An initial dose of 80 mg per
kilogram per day, in 4 or 5 divided doses is usually well tolerated and does
not cause important hepatotoxicity. It is recommended to give this dose during
the first two weeks and then taper it over the next 3 or 4 weeks. Nowadays,
other nonsteroidal antiinflammatory drugs have been used with the same
efficacy. Rest is only necessary during
the acute phase.
Carditis
Bed rest is advisable for all patients and should be
proportional to the severity of the disease and continue as long as necessary. Steroids are more
potent antiinflammatory drugs than salycilates as they can cause a more rapid
improvement of acute manifestations of carditis, although they do not alter the
course of rheumatic fever and subsequent development of rheumatic heart
disease. There is still some controversy as to whether steroids are better than
salycilates for mild carditis, but according to the guidelines for treatment of
patients with RF (47), the use of corticosteroids should be reserved for
patients with severe rheumatic carditis.
Many experts in developing
countries prefer to treat all cases of carditis (mild to severe) with steroids
and do not feel that it is necessary to add salycilates at the end of the
treatment. They suggest starting with an initial high dose (2 mg/kg / day –
maximum 60 mg/day) during the first 3 weeks and then decreasing the dose by 20%
every week. This treatment will be completed, on average, in 6 to 8 weeks and
it will be not necessary to add acetylsalicylic acid. Some authors recommend
the use of intravenous methylprednisolone in severe cases of heart failure
(48), but occasionally, surgical intervention will be indicated in patients with
refractory congestive heart failure (39).
Immunological mechanisms appear to be involved in the
pathogenesis of acute rheumatic fever but intravenous immunoglobulin did not
alter the natural history of acute RF. There was no evidence of reduction in
the extent and severity of carditis, more rapid resolution of inflammatory
activity, or decreased chronic morbidity (49).
In established rheumatic heart
disease, recurrence of carditis may present as congestive heart failure. Both
the carditis as well as congestive failure need to be treated.
Chorea
Bed rest is required only
for severe attacks to prevent injury. Several drugs have been used to treat
chorea with varying success. Since choreiform movements are known to be
influenced by emotional stress, there has been some success with sedative drugs
especially phenobarbital, diazepam, and chlorpromazine. Other drugs used
successfully are haloperidol and valproic acid.
(49-51). Recently, in a double blind
study using prednisone and placebo in 37 children with chorea, the authors
could demonstrate a significant reduction of symptoms in the first week of treatment, and this response
was maintained until the end of the study (p<0.001) (52). Medication should be given until chorea is controlled
and then gradually tapered.
Erythema
marginatum and subcutaneous nodules
These manifestations do not
require specific medication.
Treatment
of streptococcal pharyngitis and prophylaxis
It is difficulty to be sure if there is still
streptococcal infection in the throat of a patient with RF at the diagnosis.
Therefore, all patients should receive antistreptococcal antibiotics. In
developing countries, benzathine penicillin is the preferred drug, followed by
oral penicillin or erythromycin for penicillin allergic patients (Table 2).
Table 2: Streptococcal eradication
Benzathine Penicillin
·
Patients > 27
Kg: 600.000 U, IM
·
Patients < 27
Kg: 1.200.000 U, IM
Oral penicillin for 10 days
Erythromycin
·
20 mg to 40 mg/kg
in 2 to 4 divided doses for 10 days
To prevent recurrent RF, it
is essential to start a secondary prophylaxis as soon as the diagnosis is
established. Preferentially it should be done with benzathine penicillin in the
same doses used to eradicate streptococci given every 3 weeks. Other options
are oral penicillin or sulfadiazine (1 g once a day) or erythromycin for
allergic patients.
There is no agreement about
the duration of secondary prophylaxis. In some developing countries, for
patients without carditis, it can be withdrawn at the age of 18 since the
patient has received the prophylaxis for a minimum of 5 years. In patients with
carditis, it is advisable to continue prophylaxis until at least the age of 35.
There is even less evidence-based medicine and agreement in prophylaxis of
children with rheumatic fever without carditis or for post-streptococcal
arthritis (Table 3).
|
Table
3: Secondary prophylaxis |
|
Benzathine Penicillin each 21 days |
|
·
Patients
< 27 Kg: 600,000 U , IM |
|
·
Patients > 27 Kg: 1,200,000 U, IM |
|
Benoxymethylpenicillin |
|
·
250 mg twice a
day |
|
Sulfadiazine |
|
·
Patients <
27 Kg: 0,5 g/day |
|
·
Patients >
27 Kg: 1g /day |
|
|
|
Duration: |
|
·
No carditis:
until 18 years old (and minimum of 5 years) |
|
·
With carditis:
until at least 35 years old (or life long) |
OUTCOME
Recurrences of RF often have
the same clinical pattern as the previous attack. Patients with chorea or
arthritis have the best prognosis. The prognosis
is usually good with a full recovery.
Patients with chorea generally have good prognosis with a full
recovery. However, rheumatic heart
disease can be diagnosed years after the initial attack of chorea and this
possibility should be considered carefully in respect to maintaining secondary
prophylaxis. Chorea can rarely recur after an intercurrent illness, drug
exposure or pregnancy.
The outcome of carditis has
a wide range of possibilities - from total recovery (with risk of recurrences)
to death due to cardiac failure. Between
these two ends of the spectrum are various kinds of chronic RHD. Approximately
58-74% lose evidence of cardiac involvement. This occurs mainly in patients
with a single valve (mostly mitral) involvement without cardiomegaly. However,
it rarely occurs in patients who present with multiple valve involvement,
cardiomegaly and cardiac failure. Mitral stenosis develops later but it is more
precocious in developing countries. Chances of recurrence are higher (50%)
within the first 6 months of the initial attack and lessen to only 10% after 5
years.
Death is rare during the first attack but the chances
increase during recurrences, especially in patients with pre-existing heart
involvement. If the heart is spared in the first attack, it is likely to be
spared in subsequent occurrences. Patients with valvular disease should be
educated on how to avoid infective endocarditis. Specific prophylaxis should be
given before any minor or major surgical procedure, including minor suturing
and removal of tartar from teeth. (Table
4) (53).
|
Table 4:
Infective Endocarditis Prophylaxis |
|
|
|
For almost all patients, the drug is given one
hour before the procedure unless mentioned otherwise. |
|
1. Oral
Amoxicillin 50mg/kg, it may be given IM or IV. Adults: 2 gms. |
|
2. For
patients with Amoxicillin/ Ampicillin allergy: |
|
·
Oral Clindamycin 20 mg/kg. Adults: 600mg. |
|
OR |
|
·
Oral
Cephelexin or Cefadroxil 50mg/kg. Adults: 2.0gm |
|
OR |
|
·
Oral
Azithromycin or Clarithromycin 15 mg/kg. Adults: 500 mg |
|
OR |
|
·
IV Clindamycin
20mg/kg. Adults: 600 mg |
|
OR |
|
·
IV Cefazolin
2.5 mg/kg. Adults: 1.0 gm |
|
3. If gastrointestinal or genitourinary surgery is
contemplated, then the following drugs
can be given: |
|
High
risk procedure: |
|
·
IV Ampicillin/
Amoxicillin 50 mg/ kg + Gentamycin 1.5 mg/kg 30 minutes ·
before the
procedure and 6 hours after the procedure. |
|
High
risk procedure in patients with Amoxicillin allergy: |
|
·
IV Vancomycin
20 mg/kg (Adults 1gm) over 1-2 hours + IV Gentamycin 1.5
mg/kg 30 minutes before the procedure. |
|
Moderate risk surgery: |
|
·
Only IV or IM
Amoxicillin 50 mg/kg (Adults: 1gm) |
|
Moderate risk surgery in patients with Amoxicillin allergy: |
|
·
Vancomycin 20
mg/kg (Adults: 1gm) over 1-2 hours. |
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