Acute

Rheumatic
Fever
Presented by Mazhar Khan And Aditya Bali
Under the guidance of Dr. Bakyt kulumbaev
Introduction
Acute rheumatic fever (ARF) is an autoimmune inflammatory process that develops as a sequela of streptococcal infection. It can
affect the heart, joints, brain, and skin, and is thought to be an immune response to an earlier infection. ARF can develop if strep throat,
scarlet fever, and strep skin infections are not treated properly. It usually takes about 1 to 5 weeks after one of these infections for
rheumatic fever to develop. ARF has extremely variable manifestations, and no specific diagnostic test exists.
Rheumatic fever occurs after infections with a germ or bacteria called Streptococcus pyogenes or group A streptococcus. This germ
appears to trick the immune system into attacking healthy tissues in the body. These tissues become swollen or inflamed.
This abnormal reaction seems to almost always occur with strep throat or scarlet fever. Strep infections that involve other parts of the
body do not seem to trigger rheumatic fever.
The primary risk factor for rheumatic fever is failing to take steps to prevent infection from strep bacteria (as well as other
infectious microbes). This means infrequent handwashing, especially after sneezing or coughing or before eating.
Crowded places are high risk for contracting group A strep. Daycare centers, schools, and military training facilities are especially
risky.
If your healthcare provider prescribes an antibiotic to treat strep throat or scarlet fever, it's critical to follow the instructions and finish
the entire course even if you start to feel better.
Pathophysiology
• ARF is characterized by nonsuppurative inflammatory lesions of the joints, heart, subcutaneous tissue, and central nervous system.
An extensive literature search has shown that, at least in developed countries, rheumatic fever follows pharyngeal infection with
rheumatogenic group A streptococci. The risk of developing rheumatic fever after an episode of streptococcal pharyngitis has been
estimated at 0.3-3%. Investigations of rheumatic fever occurring in the aboriginal populations of Australia suggest that
streptococcal skin infections might also be associated with the development of rheumatic fever. and that group C and G
streptococci may also serve as initiating pathogens. Although several classic group A streptococcal emm types are considered to be
rheumatogenic and most likely to be associated with acute rheumatic fever, in Oceania and Hawaii, group A streptococcal strains
not traditionally associated with rheumatic fever have been found to cause the disease. This diversity of potential inciting group A
streptococcal strains also appears to be a common phenomenon in lower and middle income countries.
• Molecular mimicry accounts for the tissue injury that occurs in rheumatic fever. Both the humoral and cellular host defenses of a
genetically vulnerable host are involved. In this process, the patient's immune responses (both B- and T-cell mediated) are unable to
distinguish between the invading microbe and certain host tissues. T helper 1 and cytokine Th17 appear to be key mediators of
rheumatic heart disease. The resultant inflammation may persist well beyond the acute infection and produces the protean
manifestations of rheumatic fever.
Symptoms
These are the most common symptoms of rheumatic fever:
• Fever
• Swollen, tender, red and extremely painful joints — particularly the
knees and ankles
• Nodules (lumps under the skin)
• Red, raised, lattice-like rash, usually on the chest, back, and abdomen
• Shortness of breath and chest discomfort
• Uncontrolled movements of arms, legs, or facial muscles
• Weakness
Diagnosis
The diagnosis of RF is based on Dr. Jones’ criteria, which were recently revised (2015). The
criteria include major and minor manifestations, and risk stratification has recently been
applied to populations, dividing them into low risk and moderate- to high-risk . The major
diagnostic criteria are carditis, arthritis, chorea, erythema marginatum, and subcutaneous
nodules, whereas the minor criteria are arthralgia, hyperpyrexia, high erythrocyte
sedimentation rate (ESR), and/or high C-reactive protein (CRP), and prolonged PR
interval . To diagnose a patient with RF as a first episode of the disease, a confirmation of
two major criteria or one major and two minor criteria is required, along with evidence of
antecedent GAS infection. The diagnosis of subsequent episodes of RF requires either two
major criteria, one major and two minor criteria, or three minor criteria . The evidence of
GAS infection is confirmed by one of the following: a positive throat culture for GAS;
increasing trend anti-streptolysin O titer (ASO) readings rather than a single titer result; or a
positive rapid group A streptococcal carbohydrate antigen test in a child who clinically
suggests a high pretest probability of streptococcal pharyngitis.
Treatment
• Treatment of ARF consists of antibiotic therapy, anti-inflammatory therapy, heart failure management, and
commencement of ongoing care (secondary prevention and provision of education) . Hospitalization is advisable
for optimal management of ARF, especially for an initial episode, so that all tests can be completed, response to
therapy can be observed, and long-term preventative measures can be started with appropriate education for
the patient and their caregivers.
Goals of treatment — The six major goals of treatment are:
●Eradication of group A beta-hemolytic Streptococcus (GAS)
●Symptomatic relief of acute disease manifestations (eg, arthritis, fever)
●Manage rheumatic heart disease (RHD; eg, carditis, heart failure) if present
●Manage chorea if present
●Prophylaxis against future GAS infection to prevent progression of cardiac disease
●Provision of education for the patient and patient's caregivers
There is no therapy that slows progression of valvular damage in the setting of ARF.
Treatment
Eradication of GAS carriage — Patients with newly diagnosed ARF are started on antibiotic
therapy to eradicate group A Streptococcus (GAS) carriage. When a patient is diagnosed with ARF,
they are presumed to have an associated GAS infection (typically pharyngitis, though pyoderma
may also trigger ARF, particularly in tropical regions), even if GAS is not identified in culture.
Thus, affected patients should receive prompt antibiotic therapy to eradicate GAS carriage,
prevent ARF recurrences, and reduce the risk of developing RHD.
GAS pharyngitis — Treatment should proceed as delineated for management of GAS
pharyngitis whether or not pharyngitis is present at the time of diagnosis. Treatment is given
even if throat cultures are negative to ensure eradication of streptococci that may persist in the
upper respiratory tract.
In practice, the most convenient and sensible approach is to administer long-acting
intramuscular (IM) penicillin G benzathine, which serves two purposes:
●To eradicate GAS carriage
●As the first dose of secondary prophylaxis that is given every 21 to 28 days
Treatment
Oral alternatives, which should be used in the case of shortages
of penicillin G benzathine or in the case of penicillin allergy, are listed in
the tables. These treatment options vary somewhat from those
preferred for secondary prevention . Penicillin allergy should be verified
by history and confirmed with testing by an allergy specialist if
necessary before choosing an alternative to penicillin G benzathine.
The efficacy of penicillin in this setting is supported by landmark
controlled studies performed in the 1950s and 1960s that demonstrated
dramatic reductions in rates of ARF and risk of ARF recurrence and/or
complications in patients who were given prompt penicillin therapy. It is
also supported by decades of clinical experience.
Treatment
Household contacts — Throat cultures (or evaluation for pyoderma if that was the GAS infection that identified the patient) should
be performed on household contacts. Those with positive results should also receive a full course of antibiotic therapy, even if
asymptomatic.
Arthritis management — Antiinflammatory agents are the mainstay of symptomatic management of ARF-associated arthritis.
●First-line agents – For most patients with symptomatic ARF-associated arthritis, we suggest a nonsteroidal antiinflammatory drug
(NSAID; eg, naproxen, ibuprofen) as the first-line antiinflammatory agent because NSAIDs have a lower risk of adverse effects
compared with other agents (eg, aspirin, glucocorticoids). We typically use naproxen in this setting since it has the advantage of less
frequent dosing. Dosing for naproxen is as follows:
•For children >2 years old – 10 to 20 mg/kg/day in two divided doses given orally every 12 hours (maximum daily dose 1000 mg)
•For adults – 250 to 500 mg twice daily (maximum daily dose 1250 mg)
The efficacy of naproxen in this setting is supported by limited clinical trial data and observational studies .
Ibuprofen has been used successfully in younger children with ARF, as was shown in one small observational study.
●Duration of treatment – NSAIDs are given as a standing dose until symptoms resolve and inflammatory markers have improved,
provided the medication is well tolerated. Improvement is typically noted in one to two weeks, although longer courses (6 to 12
weeks) may be needed for persistent or rebound symptoms and elevated C-reactive protein (CRP) and erythrocyte sedimentation rate
(ESR)
Complications of ARF
• Complications of acute rheumatic fever include heart inflammation
(carditis), chorea, and arthritis.0 Cardiac involvement during acute
rheumatic fever can result in rheumatic heart disease, which can cause
heart failure and premature mortality.1 Other complications of
rheumatic heart disease include bacterial endocarditis, which is an
infection of the inner lining of the heart, and severe narrowed or
leaking heart valves.
Prognosis of ARF
The clinical events of acute rheumatic fever have been correlated with the subsequent cardiac sequelae in 441 children and adolescents
who were receiving continuous antimicrobial prophylaxis after rheumatic episodes that occurred during 1950–1957. The follow-up
examinations, which extended for a mean of 7.8 years, were performed at monthly or bimonthly intervals in an outpatient
epidemiologic research clinic, where special methods were used to improve the objectivity, precision, and standardization of the
examining physicians' cardiac auscultation and roentgenographic interpretation.
“Carditis” was defined clinically according to the presence or absence of significant cardiac murmurs, specified by rigorous criteria;
“severe carditis” was manifested by significant cardiac enlargement or decompensation. The long-term results showed the following:
1. The proportionate distribution and severity of carditic and noncarditic clinical features in the population was similar to that of a
comparable population whose acute rheumatic episodes occurred during 1958–1960.
2. All of the 181 patients who were initially free of carditis have remained free of rheumatic heart disease.
3. Of the 260 patients with various forms of carditis initially, the evidence of cardiac damage has disappeared in 113, leaving only 147
patients with definite residual rheumatic heart disease.
4. Of those 147 patients, 12 have died. All but one of these 12 patients had severe carditis in the antecedent acute rheumatic
episode, and only one had later evidence of recurrent or persistent acute rheumatic inflammation.
Prognosis of ARF
5. The disappearance of rheumatic heart disease was most likely to occur in patients who had: mild rather than severe carditis; a first
rheumatic attack rather than a recurrence; systolic murmurs only rather than diastolic murmurs; murmurs of one valve rather than of
two; and arthropathic manifestations rather than no joint symptoms.
6. In the 271 patients whose acute rheumatic episodes were first attacks, the cardiac outcome depended on the presence and
severity of carditis when treatment began, and was unrelated to the agent of treatment or to the promptness with which treatment
was started after the onset of symptoms.
7. Evidence of pericarditis was most common in patients with other manifestations of carditis and did not alone appear to influence
prognosis.
8. A prolonged P-R electrocardiographic interval occurred in about one third of all patients during the acute rheumatic episodes,
regardless of the simultaneous presence or severity of carditis, and had no direct relationship to the ultimate cardiac state.
These results confirm certain well-established concepts of clinical behavior in acute rheumatic fever, and contradict others. They
indicate the importance of careful attention to clinical observation in recognizing the different subgroups of patients, with different
cardiac prognosis, who constitute the complex, diverse spectrum of acute rheumatic fever.
prevention
• Prevention of initial development of rheumatic fever
(primary prevention) is accomplished by prompt diagnosis
and antibiotic treatment of group A Streptococcus (GAS)
tonsillopharyngitis and, in some settings, GAS pyoderma
• Appropriate antibiotic treatment of streptococcal
pharyngitis prevents ARF in most cases . However, at least
one-third of ARF episodes occur in the setting of subclinical
streptococcal infection . In addition, ARF is not preventable
in symptomatic patients who do not seek medical care.
 • The diagnosis of acute rheumatic fever
requires:
• 2 major, or
• 1 major and 2 minor manifestations, AND
• evidence of group A streptococcal infection

Jones criteria • Required Criteria

• Evidence of antecedent Strep infection:
ASO / Strep antibodies / Strep group A
throat culture / Recent scarlet fever /
anti-deoxyribonuclease B / anti-
hyaluronidase
 • Major Diagnostic Criteria
• Carditis
• Polyarthritis
• Chorea
• Erythema marginatum
• Subcutaneous Nodules

Jones criteria • Minor Diagnostic Criteria

• Fever
• Arthralgia
• Previous rheumatic fever or rheumatic heart disease
• Elevated acute phase reactant (ESR or CRP)
• Prolonged PR interval
Conclusion
• In conclusion, acute rheumatic fever is a serious condition that can have long-term
consequences if left untreated. This presentation has provided an overview of the
disease, including its causes, symptoms, and diagnostic methods. Early diagnosis and
appropriate treatment are crucial in preventing complications and managing the
condition effectively. It is important to raise awareness about acute rheumatic fever
among healthcare professionals, as well as the general public, to promote timely
diagnosis, appropriate treatment, and prevention strategies. Through ongoing research,
education, and public health initiatives, we can work towards reducing the burden of
acute rheumatic fever and its associated complications.