History and exam
Key diagnostic factors
common
fever
Occurs in nearly all cases at the onset of the illness. Historically, most cases will have fever above 39°C (102.2°F), but many experts suggest that 38°C (100.4°F) measured orally, rectally, or tympanic is significant.
The Jones criteria also recognise fever as a minor manifestation of disease: ≥38.5°C (≥101.3°F; low-risk populations) or ≥38.0°C (≥100.4°F; moderate- to high-risk populations).[2]
joint pain
The pain is often extreme, and if the lower limbs are affected the patient often cannot walk. The most commonly affected joints are the knees, ankles, wrists, elbows, and hips. Typically asymmetrical and usually but not always migratory. A joint may be affected for a period of hours or a couple of days.
If the patient has monoarthritis and is suspected to have acute rheumatic fever (ARF), but does not meet the criteria for diagnosis, the patient should withhold from treatment with non-steroidal anti-inflammatory drugs (NSAIDs) so that the appearance of migratory polyarthritis (a major manifestation) is not masked.
The arthritis of ARF is very sensitive to salicylates such as aspirin (as well as other NSAIDs), and joint symptoms typically respond within several days of treatment with anti-inflammatory drugs.
Other diagnostic factors
common
recent sore throat or scarlet fever
May suggest recent group A streptococcal pharyngitis.
recent skin infection
May suggest recent group A streptococcal pyoderma.
chest pain
This can be a symptom of severe carditis.
shortness of breath
This can be a symptom of severe carditis.
palpitations
Individuals with rheumatic carditis may experience palpitations in association with advanced heart block. Those with longstanding rheumatic heart disease and left atrial dilatation are also predisposed to atrial fibrillation.
heart murmur
Mitral regurgitation is the most common clinical manifestation of carditis as a pan-systolic murmur heard loudest at apex, radiating to axilla, and accentuated when the patient is rolled over on their left side.
A Carey-Coombs murmur may be heard, caused by pseudo-mitral stenosis because of the increased flow of the regurgitant volume of blood over the mitral valve during diastolic filling of the left ventricle.
Aortic valvulitis manifests as aortic regurgitation and is characterised by an early diastolic murmur heard at the base of the heart, accentuated by the patient sitting forward in held expiration.
The regurgitant murmurs are heard more commonly in children and young adolescents.
Chronic rheumatic mitral stenosis produces an apical diastolic murmur, a low-pitched mid-diastolic rumble that may be preceded by an opening snap.
Auscultation sounds: Mitral regurgitation (severe)
Auscultation sounds: Aortic regurgitation (severe)
Auscultation sounds: Mitral stenosis (severe)
pericardial rub
If there is pericardial involvement during the acute phase of acute rheumatic fever, a pericardial rub may be heard.
signs of cardiac failure
Patients with severe carditis may exhibit signs of congestive heart failure due to severe regurgitation and impaired cardiac function.
swollen joints
Joint swelling, accompanied by tenderness, warmth, and restricted movement, indicates arthritis rather than arthralgia. The most commonly affected joints are the knees, ankles, wrists, elbows, and hips. Detection of arthritis of the hip joint can be more difficult than detection of arthritis involving other large joints, and careful attention should be paid to limitation of movement and inability to bear weight. The arthritis of acute rheumatic fever (ARF) is typically asymmetrical and usually but not always migratory. A joint may be affected for a period of hours or a couple of days. Additional joints may flare up as one or others improve.
The arthritis of ARF is very sensitive to salicylates such as aspirin (as well as other non-steroidal anti-inflammatory drugs), and joint symptoms usually respond within several days of treatment with these anti-inflammatory drugs. Some patients may present with pain and swelling affecting a single joint (monoarthritis). Polyarthralgia is defined as arthritic pain and aches in the joints, joint pains, arthralgia of multiple joints, and multiple joint pain. Arthralgia is a criterion in the revised Jones criteria.
uncommon
restlessness
May suggest chorea. Chorea is more common in females and may occur up to 6 months after group A streptococcal throat infection. Chorea always disappears with sleep, and is made more pronounced by purposeful movements.
clumsiness
May suggest chorea, which more commonly affects females after a latency period of up to 6 months after group A streptococcal throat infection. Chorea always disappears with sleep, and is made more pronounced by purposeful movements.
emotional lability and personality changes
May suggest chorea, which typically affects females after a latency period of up to 6 months after group A streptococcal infection. Chorea always disappears with sleep, and is made more pronounced by purposeful movements.
jerky, uncoordinated choreiform movements
Can affect the whole body, or just one side of the body (hemi-chorea). The head is often involved, with erratic movements of the face that resemble grimaces, grins, and frowns. Chorea always disappears with sleep, and is made more pronounced by purposeful movements.
inability to maintain protrusion of the tongue
Consistent with chorea affecting the tongue. May resemble a 'bag of worms' when protruded.
milkmaid's grip
Rhythmic squeezing when the patient grasps the examiner's hands, consistent with chorea.
spooning sign
Flexion of the wrists and extension of the fingers when the hands are extended, seen with chorea.
pronator sign
Turning outwards of the arms and palms when held above the head, seen with chorea.
erythema marginatum
Pink serpiginous rash with a well-defined edge. It begins as a macule and expands with central clearing. The rash may appear and then disappear before the examiner's eyes, leading to the descriptive term of patients having 'smoke rings' beneath the skin. Rare, occurring in <5% of cases. Usually appears during the acute phase of rheumatic fever but may recur for weeks or even months after the acute phase has subsided. Difficult to detect in dark-skinned people.[Figure caption and citation for the preceding image starts]: Typical appearance of erythema marginatum on the back of a child with acute rheumatic feverCourtesy of Professor Mike South, Royal Children’s Hospital Melbourne; used with permission [Citation ends].
May be accentuated by warmth and so is often noted during a bath or shower.
subcutaneous nodules
Firm and painless lumps, 0.5 cm to 2 cm in diameter, found mainly over the extensor surfaces or bony protuberances, particularly on the extensor surfaces of the elbows, hands, feet, and over the occiput and upper back. Rare, occurring in <5% of cases. Often appear after the onset of acute rheumatic fever and last from a few days to 3 weeks.
pregnancy or taking oral contraceptive pill
May be associated with recurrence of rheumatic chorea. Increased cardiac demands during pregnancy may also lead to deterioration in cardiac status. In particular, pregnant women with rheumatic mitral stenosis may develop breathlessness, fatigue, or palpitations.
Risk factors
strong
poverty
Acute rheumatic fever is a disease of poverty. The reduction in rates of rheumatic fever in high-income countries was mostly due to changes in socioeconomic status and access to health care.[10] Although there are conflicting data from prevalence surveys regarding the association between socioeconomic indicators and rheumatic heart disease (RHD), most surveys do confirm an association with low socioeconomic conditions. For example, in a prevalence survey of RHD in children in Rajasthan, India, the prevalence of RHD in the lowest socioeconomic status group was 3.9 in 1000, in the middle socioeconomic status group it was 2.1 in 1000, and in the highest socioeconomic status group no cases of RHD were detected.[36]
An ecological study from New Zealand that matched hospitalisation data with census area unit data (summated data for population parcels of approximately 5000 people) found rheumatic fever rates in the most crowded quintile to be 23 times greater than that in the least crowded quintile.[37]
overcrowded living quarters
Perhaps the most important environmental factor is household overcrowding.[38][39] The classical studies during the 1950s in US Air Force Base barracks found that rates of acquisition of streptococcal infections increased when beds were moved closer together, thus providing a biological basis for a relationship between overcrowding and the incidence of acute rheumatic fever.[40] Household crowding has been shown to be strongly associated with rheumatic fever in New Zealand.[37]
family history of rheumatic fever
An inherited susceptibility to acute rheumatic fever (ARF) is likely. Familial clustering of cases of ARF has been described.[16] Earlier twin studies found weak concordance in monozygotic twins,[41] but a more recent meta-analysis found that the risk of rheumatic fever in a monozygotic twin with a history of rheumatic fever in the co-twin was 6 times greater than in dizygotic twins, suggesting that rheumatic fever is a disorder with a high heritability.[17]
HLA association
Particular HLA class II alleles appear to be associated with rheumatic fever susceptibility. HLA DRB1, DQA1, and DQB1 haplotypes were associated with rheumatic heart disease in Egyptian populations.[42]
genetic susceptibility
Genome-wide association studies have identified susceptibility signals in immune loci. In Oceania populations, an association has been observed between one common immunoglobulin heavy chain allele (IGHV4-61*02) and rheumatic heart disease (RHD), with each copy of IGHV4-61*02 associated with a 1.4-fold increase in the risk of RHD.[26] A second study found that variation at HLA-DQA1-DQB1 was the major genetic risk factor for RHD among Aboriginal Australians.[27]
Among peoples of Maori and Pacific ancestry in New Zealand, polymorphisms on the IL-6 gene have been shown to be associated with increased incidence of RHD.[43] A multicentre study across 8 African countries identified a single RHD risk locus, 11q24.1, which reached genome-wide significance in black African individuals.[28] Further multi-country data have solidified the genetic hypothesis associated with developing RHD and unique proteomic signatures associated with severe RHD have been identified.[44]
indigenous populations; Aboriginal Australian, Asian, and Pacific Islanders
Particular racial and ethnic groups appear to be at higher risk of acute rheumatic fever than others. This is particularly evident in countries where indigenous populations are at increased risk compared with non-indigenous populations, including Maori children in New Zealand, Aboriginal children in Australia, and Polynesian children in Hawaii.[22][23][24][25] This appears to be a combination of environmental factors such as overcrowding, poor access to health care (primary prevention), and an underlying inherited susceptibility.
weak
D8/17 B cell antigen positivity
The D8/17 antigen is expressed on B cells and may act as a binding site for group A streptococci on these cells.[45] There is a strong association in a number of populations between the expression of D8/17 antigen and rheumatic fever, including in the US, Australia, Israel, Russia, Mexico, and Chile.[18][19][20] However, this association is not universal as there has been no association found in other populations including in the US,[21] and less strong associations in populations in India.[46]
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