Rheumatic fever

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Rheumatic Heart Disease
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Rheumatic fever
Other namesAcute rheumatic fever (ARF)
myocardium)
SpecialtyCardiology
SymptomsFever, multiple painful joints, involuntary muscle movements, erythema marginatum[1]
ComplicationsRheumatic heart disease, heart failure, atrial fibrillation, infection of the valves[1]
Usual onset2–4 weeks after a streptococcal throat infection, age 5–14 years[2]
CausesAutoimmune disease triggered by Streptococcus pyogenes[1]
Risk factorsGenetics, malnutrition, poverty[1]
Diagnostic methodBased on symptoms and infection history[3]
PreventionAntibiotics for strep throat, improved sanitation[1][4]
TreatmentProlonged periods of antibiotics, valve replacement surgery, valve repair[1]
Frequency325,000 children a year[1]
Deaths319,400 (2015)[5]

Rheumatic fever (RF) is an inflammatory disease that can involve the heart, joints, skin, and brain.[1] The disease typically develops two to four weeks after a streptococcal throat infection.[2] Signs and symptoms include fever, multiple painful joints, involuntary muscle movements, and occasionally a characteristic non-itchy rash known as erythema marginatum.[1] The heart is involved in about half of the cases.[1] Damage to the heart valves, known as rheumatic heart disease (RHD), usually occurs after repeated attacks but can sometimes occur after one.[1] The damaged valves may result in heart failure, atrial fibrillation and infection of the valves.[1]

Rheumatic fever may occur following an infection of the throat by the bacterium Streptococcus pyogenes.[1] If the infection is left untreated, rheumatic fever occurs in up to three percent of people.[6] The underlying mechanism is believed to involve the production of antibodies against a person's own tissues.[1] Due to their genetics, some people are more likely to get the disease when exposed to the bacteria than others.[1] Other risk factors include malnutrition and poverty.[1] Diagnosis of RF is often based on the presence of signs and symptoms in combination with evidence of a recent streptococcal infection.[3]

Treating people who have strep throat with

developing world.[1] Other preventive measures include improved sanitation.[1] In those with rheumatic fever and rheumatic heart disease, prolonged periods of antibiotics are sometimes recommended.[1] Gradual return to normal activities may occur following an attack.[1] Once RHD develops, treatment is more difficult.[1] Occasionally valve replacement surgery or valve repair is required.[1] Otherwise complications are treated as usual.[1]

Rheumatic fever occurs in about 325,000 children each year and about 33.4 million people currently have rheumatic heart disease.

rheumatic disorders.[11]

Signs and symptoms

A culture-positive case of streptococcal pharyngitis with typical tonsillar exudate in a 16-year-old

The disease typically develops two to four weeks after a

throat infection.[2] Symptoms include: fever, painful joints with those joints affected changing with time, involuntary muscle movements, and occasionally a characteristic non-itchy rash known as erythema marginatum. The heart is involved in about half of the cases. Damage to the heart valves usually occurs only after several attacks but may occasionally occur after a single case of RF. The damaged valves may result in heart failure and also increase the risk of atrial fibrillation and infection of the valves.[1]

Pathophysiology

Rheumatic fever is a

spore forming, and forms chains and large colonies.[14]

S. pyogenes has a

macrophages.[16]

In rheumatic fever, these lesions can be found in any layer of the heart causing different types of

MacCallum plaques.[citation needed
]

Rheumatic heart disease

Pathophysiology of rheumatic heart disease
Micrograph showing an Aschoff body (right of image), as seen in rheumatic heart disease. H&E stain

Chronic rheumatic heart disease (RHD) is characterized by repeated inflammation with fibrinous repair. The cardinal anatomic changes of the valve include leaflet thickening, commissural fusion, and shortening and thickening of the tendinous cords.

autoimmune reactions.[20]

Molecular mimicry occurs when epitopes are shared between host antigens and Streptococcus antigens.[21] This causes an autoimmune reaction against native tissues in the heart that are incorrectly recognized as "foreign" due to the cross-reactivity of antibodies generated as a result of epitope sharing. The valvular endothelium is a prominent site of lymphocyte-induced damage. CD4+ T cells are the major effectors of heart tissue autoimmune reactions in RHD.[22] Normally, T cell activation is triggered by the presentation of bacterial antigens. In RHD, molecular mimicry results in incorrect T cell activation, and these T lymphocytes can go on to activate B cells, which will begin to produce self-antigen-specific antibodies. This leads to an immune response attack mounted against tissues in the heart that have been misidentified as pathogens. Rheumatic valves display increased expression of VCAM-1, a protein that mediates the adhesion of lymphocytes.[23] Self-antigen-specific antibodies generated via molecular mimicry between human proteins and streptococcal antigens up-regulate VCAM-1 after binding to the valvular endothelium. This leads to the inflammation and valve scarring observed in rheumatic valvulitis, mainly due to CD4+ T cell infiltration.[23]

While the mechanisms of genetic predisposition remain unclear, a few genetic factors have been found to increase susceptibility to autoimmune reactions in RHD. The dominant contributors are a component of

mitral valve stenosis has been associated with MBL2 alleles encoding for high production of MBL.[26] Aortic valve regurgitation in RHD patients has been associated with different MBL2 alleles that encode for low production of MBL.[27] In addition, the allele IGHV4-61, located on chromosome 14, which helps code for the immunoglobulin heavy chain (IgH) is linked to greater susceptibility to RHD because it may affect protein structure of the IgH.[28] Other genes are also being investigated to better understand the complexity of autoimmune reactions that occur in RHD.[citation needed
]

Diagnosis

Synovial fluid examination[29][30]
Type WBC (per mm3) % neutrophils Viscosity Appearance
Normal <200 0 High Transparent
Osteoarthritis <5000 <25 High Clear yellow
Trauma <10,000 <50 Variable Bloody
Inflammatory 2,000–50,000 50–80 Low Cloudy yellow
Septic arthritis >50,000 >75 Low Cloudy yellow
Gonorrhea ~10,000 60 Low Cloudy yellow
Tuberculosis ~20,000 70 Low Cloudy yellow
Inflammatory: Arthritis, gout, rheumatoid arthritis, rheumatic fever
Streptococcus pyogenes bacteria (Pappenheim's stain), the trigger for rheumatic fever

The original method of diagnosing rheumatic heart disease was through heart auscultation, specifically listening for the sound of blood regurgitation from possibly dysfunctional valves. However, studies have shown that

indolent carditis, each of which by itself can indicate rheumatic fever.[37][38][39] An April 2013 review article in the Indian Journal of Medical Research stated that echocardiographic and Doppler (E & D) studies, despite some reservations about their utility, have identified a massive burden of rheumatic heart disease, which suggests the inadequacy of the 1992 Jones' criteria. E & D studies have identified subclinical carditis in patients with rheumatic fever, as well as in follow-ups of rheumatic heart disease patients who initially presented as having isolated cases of Sydenham's chorea.[40] Signs of a preceding streptococcal infection include: recent scarlet fever, raised antistreptolysin O or other streptococcal antibody titre, or positive throat culture.[41] The last revision of 2015 suggested variable diagnostic criteria in low-risk and high-risk populations to avoid overdiagnosis in the first category and underdiagnosis in the last one.[34] Low-risk populations were defined as those with acute rheumatic fever annual incidence ≤2 per 100 000 school-aged children or all-age rheumatic heart disease prevalence of ≤1 per 1000.[34] All other populations were categorised as having a moderate or high risk.[34]

Major criteria

  1. Joint manifestations are the unique clinical signs that have different implications for different population-risk categories : Only polyarthritis[42] (a temporary migrating inflammation of the large joints, usually starting in the legs and migrating upwards) is considered as a major criterion in low-risk populations, whereas monoarthritis, polyarthritis and polyarthralgia (joint pain without swelling) are all included as major criteria in high-risk populations.[34]
  2. Carditis: Carditis can involve the pericardium (pericarditis which resolves without sequelae), some regions of the myocardium (which might not provoke systolic dysfunction), and more consistently the endocardium in the form of valvulitis.[43] Carditis is diagnosed clinically (palpitations, shortness of breath, heart failure, or a new heart murmur) or by echocardiography/Doppler studies revealing mitral or aortic valvulitis. Both of clinical and subclinical carditis are now considered a major criterion.[34][43]
  3. Subcutaneous nodules: Painless, firm collections of collagen fibers over bones or
    tendons. They commonly appear on the back of the wrist, the outside elbow, and the front of the knees.[citation needed
    ]
  4. macules, which spread outward and clear in the middle to form rings, which continue to spread and coalesce with other rings, ultimately taking on a snake-like appearance. This rash typically spares the face and is made worse with heat.[citation needed
    ]
  5. Sydenham's chorea (St. Vitus' dance): A characteristic series of involuntary rapid movements of the face and arms. This can occur very late in the disease for at least three months from onset of infection.[citation needed]

Minor criteria

  1. Arthralgia: Polyarthralgia in low-risk populations and monoarthralgia in others.[34] However, joint manifestations cannot be considered in both major and minor categories in the same patient.[34]
  2. Fever: ≥ 38.5 °C (101.3 °F) in low-incidence populations and ≥ 38 °C (100.4 °F) in high-risk populations.[34]
  3. Raised
    C reactive protein (>3.0 mg/dL).[34]
  4. ECG showing a prolonged PR interval[34][41][44]
    after accounting for age variability (Cannot be included if carditis is present as a major symptom)

Prevention

Rheumatic fever can be prevented by effectively and promptly treating

domestic air pollution[47]
have been cited as associated risk factors.

In those who have previously had rheumatic fever, antibiotics may be used in a preventative manner as

secondary prophylaxis.[45] Antibiotic prophylaxis after an episode of acute rheumatic fever is recommended owing to the high likelihood of recurrence.[48] Streptococcal pharyngitis may occur asymptomatically and rheumatic fever may recur even after a treated infection.[49] The American Heart Association recommends, based on low quality evidence but with high predicted efficacy, that people with mitral stenosis due to rheumatic heart disease receive prophylactic antibiotics for 10 years or until age 40, whichever would be longer.[49] The AHA also supports good dental hygiene in people with RHD, and antibiotics for the prevention of infective endocarditis during dental procedures are recommended in high-risk patients.[49]

Vaccine

No vaccines are currently available to protect against S. pyogenes infection, although research is underway to develop one.[50] Difficulties in developing a vaccine include the wide variety of strains of S. pyogenes present in the environment and the large amount of time and number of people that will be needed for appropriate trials for safety and efficacy of the vaccine.[51]

Treatment

The management of rheumatic fever is directed toward the reduction of inflammation with

anti-inflammatory medications such as aspirin or corticosteroids. Individuals with positive cultures for strep throat should also be treated with antibiotics.[42]

Infection

People with positive cultures for Streptococcus pyogenes should be treated with penicillin as long as allergy is not present. The use of antibiotics will not alter cardiac involvement in the development of rheumatic fever.[42] Some suggest the use of benzathine benzylpenicillin.[citation needed]

Monthly injections of long-acting penicillin must be given for a period of five years in patients having one attack of rheumatic fever. If there is evidence of carditis, the length of therapy may be up to 40 years. Another important cornerstone in treating rheumatic fever includes the continual use of low-dose antibiotics (such as penicillin, sulfadiazine, or erythromycin) to prevent recurrence.[citation needed]

Inflammation

Aspirin at high doses has historically been used for treatment of rheumatic fever.

salicylate poisoning, necessitating serum monitoring of salicylate levels, and the risk of Reye syndrome, a serious and potentially deadly condition that may arise in children treated with aspirin or aspirin-containing products, alternatives to aspirin have been sought, especially in children.[48] While evidence suggests that treatment of rheumatic fever–associated arthritis with naproxen may be equally effective as with aspirin,[48][53] its role in managing carditis has not been established.[54] Management of carditis in acute rheumatic fever is controversial and based on dated literature.[55] Corticosteroids may be considered, especially in people with allergies to NSAIDs or severe disease,[48] although use of steroids may cause tissue atrophy, which could present challenges during future cardiac surgery for valve repair.[55]

Heart failure

Some patients develop significant

congestive heart failure. This requires the usual treatment for heart failure: ACE inhibitors, diuretics, beta blockers, and digoxin.[citation needed] Unlike typical heart failure, rheumatic heart failure responds well to corticosteroids.[citation needed
]

Epidemiology

Deaths from rheumatic heart disease per million persons in 2012
  0–7
  8–14
  15–20
  21–25
  26–32
  33–38
  39–45
  46–52
  53–63
  64–250
Disability-adjusted life year for rheumatic heart disease per 100,000 inhabitants in 2004.[56]
  no data
  less than 20
  20–40
  40–60
  60–80
  80–100
  100–120
  120–140
  140–160
  160–180
  180–200
  200–330
  more than 330

About 33 million people are affected by rheumatic heart disease with an additional 47 million having asymptomatic damage to their heart valves.[46] As of 2010 globally it resulted in 345,000 deaths, down from 463,000 in 1990.[57]

In Western countries, rheumatic fever has become fairly rare since the 1960s, probably due to the widespread use of antibiotics to treat streptococcus infections. While it has been far less common in the United States since the beginning of the 20th century, there have been a few outbreaks since the 1980s.[58] The disease is most common among Indigenous Australians (particularly in central and northern Australia), Māori, and Pacific Islanders, and is also common in Sub-Saharan Africa, Latin America, the Indian subcontinent, and North Africa.[59]

Rheumatic fever primarily affects children between ages 5 and 17 years and occurs approximately 20 days after strep throat. In up to a third of cases, the underlying strep infection may not have caused any symptoms.[citation needed]

The rate of development of rheumatic fever in individuals with untreated strep infection is estimated to be 3%. The incidence of recurrence with a subsequent untreated infection is substantially greater (about 50%).[60] The rate of development is far lower in individuals who have received antibiotic treatment. Persons who have had a case of rheumatic fever have a tendency to develop flare-ups with repeated strep infections.[citation needed]

The recurrence of rheumatic fever is relatively common in the absence of maintenance of low dose antibiotics, especially during the first three to five years after the first episode. Recurrent bouts of rheumatic fever can lead to valvular heart disease. Heart complications may be long-term and severe, particularly if valves are involved. In countries in Southeast-Asia, sub-Saharan Africa, and Oceania, the percentage of people with rheumatic heart disease detected by listening to the heart was 2.9 per 1000 children and by echocardiography it was 12.9 per 1000 children.[61][62][63][64] To assist in the identification of RHD in low resource settings and where prevalence of GAS infections is high, the World Heart Federation has developed criteria for RHD diagnosis using echocardiography, supported by clinical history if available.[65] The WHF additionally defines criteria for use in people younger than age 20 to diagnose "borderline" RHD, as identification of cases of RHD among children is a priority to prevent complications and progression.[46] However, spontaneous regression is more likely in borderline RHD than in definite cases, and its natural history may vary between populations.[46]

Echocardiographic

antibiotic resistance, which might be offset through use of narrow-spectrum antibiotics like benzathine benzapenicillin.[67]
Public health research is ongoing to determine if screening is beneficial and cost effective.

See also

  • Rapid strep test
  • Chronic post–RF arthropathy – joint changes that may arise following multiple episodes of rheumatic fever, also called Jaccoud's arthropathy[68]

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External links
Retrieved from "https://en.wikipedia.org/w/index.php?title=Rheumatic_fever&oldid=1214943093"