Rheumatic fever is a systemic inflammatory disease that may develop as a delayed reaction to an infection with group A beta-hemolytic streptococci. It is most often seen in children and young adults.
Presentation
Rheumatic fever does not present with specific signs and symptoms. There is sudden onset of fever, joint pain, malaise and loss of appetite. In some cases, epistaxis and abdominal pain may also be present.
The diagnosis is made with the help of Duckett Jones criteria [5]. The presence of two major criteria; or one major and two minor criteria is diagnostic.
The major criteria include the following:
- Carditis: Carditis is suggested by the presence of a number of signs that include sinus tachycardia, murmurs of mitral or aortic regurgitation, Carey-Coomb’s murmur, pericardial friction or rub, congestive heart failure, cardiomegaly and ectrocardiographic abnormalities.
- Polyarthritis: Migratory polyarthritis is present in up to 75% of the cases and mainly involves large joints such as knees, ankles, elbows and wrists.
- Syndenham’s chorea: Syndenham’s chorea develops in less than 10% of the patients suffering from rheumatic fever. However, when it is present, it is the most diagnostic feature of rheumatic fever.
- Erythema marginatum: Pink rashes with slightly raised edges may develop on the trunk or limbs.
- Subcutaneous nodules: Small, hard, painless nodules may form under the skin.
The minor criteria include the following:
- Fever
- Arthralgia
- Previous history of rheumatic fever
- Raised Erythrocyte Sedimentation Rate (ESR) or C-reactive protein (CRP)
- Leukocytosis
- Prolonged PR interval on electrocardiogram
Entire Body System
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Fever
Streptococcus - rheumatic fever; Strep throat - rheumatic fever; Streptococcus pyogenes - rheumatic fever; Group A streptococcus - rheumatic fever Low DE. Nonpneumoccal streptococcal infections and rheumatic fever. In: Goldman L, Schafer AI, eds. [nlm.nih.gov]
The minor criteria include the following: Fever Arthralgia Previous history of rheumatic fever Raised Erythrocyte Sedimentation Rate (ESR) or C-reactive protein (CRP) Leukocytosis Prolonged PR interval on electrocardiogram The diagnosis of rheumatic fever [symptoma.com]
Clinical manifestations and time course of acute rheumatic fever are shown in the image below. Clinical manifestations and time course of acute rheumatic fever. [emedicine.com]
Jones criteria (revised) for guidance in the diagnosis of rheumatic fever. Circulation 1965; 32: 664–668. Google Scholar 4. Committee on rheumatic fever and bacterial endocarditis of the American Heart Association. [doi.org]
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Congestive Heart Failure
infective endocarditis, stroke, and congestive heart failure). Antibiotics can prevent acute rheumatic fever if administered no more than 9 days after symptom onset. [ncbi.nlm.nih.gov]
Other medications may be necessary to handle congestive heart failure. If heart valve damage occurs, surgical repair or replacement of the valve may be considered. [chw.org]
The most advanced condition is congestive heart failure. Diagnosis and Treatment Blood tests may detect a high white blood cell count and a high erythrocyte sedimentation rate. [hon.ch]
failure, cardiomegaly and ectrocardiographic abnormalities. [symptoma.com]
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Fatigue
Symptoms of rheumatic fever include: Fever Abdominal pain Fatigue Heart palpitations Chest pain Shortness of breath Joints that are tender, painful, red or swollen (mainly the knees, ankles, elbows and wrists) Painless rash with jagged edges (erythema [wellstar.org]
Symptoms include: Fever Pain in one joint that migrates to another joint Red, hot or swollen joints Small, painless nodules beneath the skin Rapid, fluttering or pounding heartbeats (palpitations) Fatigue Shortness of breath A painless rash with a ragged [parentcenterhub.org]
They may include: Fever Painful and tender joints, most often the ankles, knees, elbows or wrists Pain that moves from joint to joint Red, hot or swollen joints Small, painless nodules under the skin Chest pain Heart murmur Fatigue Flat or slightly raised [arthritis.org]
Inflammation of the heart can lead to chest pain, palpitations, a sensation that the heart is fluttering or pounding hard, panting, and shortness of breath, and fatigue. [medicalnewstoday.com]
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Malaise
There is sudden onset of fever, joint pain, malaise and loss of appetite. In some cases, epistaxis and abdominal pain may also be present. The diagnosis is made with the help of Duckett Jones criteria. [symptoma.com]
Clinical Features of Acute Rheumatic Fever: Musculoskeletal Polyarthritis Arthralgia (often migratory) and legs first Poor co-ordination Non-Musculoskeletal Systemic features - sore throat before onset of joint pains, fever, pallor, weight loss, anorexia, malaise [pmmonline.org]
Symptoms Symptoms of ARF include: fever malaise (feeling of being unwell) painful and/or swollen joints (one or many joints) – usually affecting the elbows, wrists, hips, knees and ankles chest pain, difficulty breathing, or a rapid heartbeat and heart [sahealth.sa.gov.au]
After an incubation period of 2-4 days, the invading organisms elicit an acute inflammatory response, with 3-5 days of sore throat, fever, malaise, headache, and elevated leukocyte count. [emedicine.com]
It can cause streptococcal sore throat which is characterized by fever, enlarged tonsils, tonsillar exudate, sensitive cervical lymph nodes and malaise ( 6, 9 ). If untreated, strep throat can last 7-10 days ( 9 ). [phac-aspc.gc.ca]
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Anemia
Mild to moderate normocytic, normochromic anemia may be present. Chest X-ray: Chest X-ray will either be normal or will show signs of cardiomegaly, pulmonary edema and/or increased pulmonary vascularity. [symptoma.com]
It is important that your doctor make sure that your symptoms aren't caused by gout, sickle cell anemia, leukemia, embolic bacterial endocarditis, drug reactions or arthritis, all of which can have symptoms similar to those of rheumatic fever. [cedars-sinai.org]
Affected individuals may also develop leukocytosis, moderate anemia, and proteinuria. C-reactive protein, evaluated in a specimen of blood, is abnormally high in concentration. Recurrences of rheumatic fever are common. [medical-dictionary.thefreedictionary.com]
Abdominal pain, rapid sleeping pulse rate, tachycardia out of proportion to fever, malaise, anemia, leukocytosis, epistaxis, and precordial pain also may be noted in patients with ARF. [dx.doi.org]
Respiratoric
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Cough
Abstract We present a case of heterogeneous and strongly increased myocardial and valvular 18F-FDG uptake on 18F-FDG PET/CT in an HIV-positive patient with productive cough, fever, weight loss, and progressive dyspnea for 6 months. [ncbi.nlm.nih.gov]
So remember when coughing and sneezing cover your mouth and wash and dry your hands often, especially after coughing and sneezing. [Video of Dr Sciascia and Tofiga Fepulea’i in the clinic. [health.govt.nz]
Strep throat is infectious and can spread through cough and sneeze droplets. [spasifikmag.com]
To stop "strep throat" from spreading you need to cover your mouth when coughing or sneezing. You can cough and sneeze while you are asleep too. [lakesdhb.govt.nz]
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Dyspnea
Abstract We present a case of heterogeneous and strongly increased myocardial and valvular 18F-FDG uptake on 18F-FDG PET/CT in an HIV-positive patient with productive cough, fever, weight loss, and progressive dyspnea for 6 months. [ncbi.nlm.nih.gov]
[…] carditis occurs after months chorea presents after months risk of recurrence decreases with time Presentation Symptoms migratory joint pains, especially in the knees, ankles, and elbows chest discomfort heart failures (rare) Physical exam fever may have dyspnea [step2.medbullets.com]
In this setting, symptoms may include dyspnea, orthopnea, paroxysmal nocturnal dyspnea, cough, and even hemoptysis. Pulmonary venous hypertension may lead to pulmonary hypertension and right heart failure. [clinicaladvisor.com]
Gastrointestinal
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Abdominal Pain
In some cases, epistaxis and abdominal pain may also be present. The diagnosis is made with the help of Duckett Jones criteria. The presence of two major criteria; or one major and two minor criteria is diagnostic. [symptoma.com]
Clinical presentation Signs and symptoms of rheumatic fever include fever, migratory arthritis, abdominal pain, erythema marginatum (a ring-shaped rash located on trunk and upper parts of arms and legs), Sydenham's chorea, subcutaneous nodules, epistaxis [web.archive.org]
Other clinical manifestations Abdominal pain: Abdominal pain usually occurs at the onset of acute RF, resembles other conditions with acute microvascular mesenteric inflammation, and may mimic acute appendicitis. [slideshare.net]
Symptoms of rheumatic fever include: Fever Abdominal pain Fatigue Heart palpitations Chest pain Shortness of breath Joints that are tender, painful, red or swollen (mainly the knees, ankles, elbows and wrists) Painless rash with jagged edges (erythema [wellstar.org]
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Loss of Appetite
There is sudden onset of fever, joint pain, malaise and loss of appetite. In some cases, epistaxis and abdominal pain may also be present. The diagnosis is made with the help of Duckett Jones criteria. [symptoma.com]
[…] of appetite and body weight. [britannica.com]
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Epigastric Pain
A 45-year-old hypertensive Japanese woman presented with epigastric pain on inspiration, fever, complete atrioventricular block and polyarthritis. Her antistreptolysin O levels were markedly elevated. [ncbi.nlm.nih.gov]
Cardiovascular
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Heart Disease
Echocardiographic examination of children with rheumatic fever for rheumatic heart disease may over diagnose rheumatic heart disease unless congenital mitral valve anomalies and physiological regurgitation are excluded. [ncbi.nlm.nih.gov]
Characteristics, complications, and gaps in evidence-based interventions in rheumatic heart disease: the Global Rheumatic Heart Disease Registry (the REMEDY study). Eur Heart J. 2014. Google Scholar 136. [dx.doi.org]
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Chest Pain
A previously healthy, 9-year-old girl presented to the emergency department with complaints of sore throat, low-grade fever, and intermittent chest pain. She was found to have a positive rapid streptococcal antigen test. [ncbi.nlm.nih.gov]
Symptoms of rheumatic heart disease include: dizziness chest pain shortness of breath tiredness It can take many years for these symptoms to develop after a previous episode of rheumatic fever. [nhsdirect.wales.nhs.uk]
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Heart Murmur
CONCLUSION: Heart murmur, arthritis, arthralgia with reduced physical activity and chorea are the main clinical reasons that lead to suspicion in the ARF group. [ncbi.nlm.nih.gov]
A heart murmur of MR or AI indicates valvulitis. Pericarditis may present with chest pain, friction rub, pericardial effusion and EKG changes. Signs of CHF may be present. Chorea. [web.archive.org]
Echocardiography/Doppler findings not consistent with carditis should exclude that diagnosis in patients with a heart murmur otherwise thought to indicate rheumatic carditis (Class I, Level of Evidence B). [acc.org]
They may include: Fever Painful and tender joints, most often the ankles, knees, elbows or wrists Pain that moves from joint to joint Red, hot or swollen joints Small, painless nodules under the skin Chest pain Heart murmur Fatigue Flat or slightly raised [arthritis.org]
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Tachycardia
The junctional tachycardia and other electrocardiogram abnormalities resolved during follow-up. [ncbi.nlm.nih.gov]
Occuring in 50% of patients with ARF, carditis presents with tachycardia out of proportion to the fever. A heart murmur of MR or AI indicates valvulitis. Pericarditis may present with chest pain, friction rub, pericardial effusion and EKG changes. [web.archive.org]
The major criteria include the following: Carditis: Carditis is suggested by the presence of a number of signs that include sinus tachycardia, murmurs of mitral or aortic regurgitation, Carey-Coomb’s murmur, pericardial friction or rub, congestive heart [symptoma.com]
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Cardiomegaly
Chest X-ray: Chest X-ray will either be normal or will show signs of cardiomegaly, pulmonary edema and/or increased pulmonary vascularity. [symptoma.com]
A 7-y-old girl presented with prolonged fever, arrhythmia and cardiomegaly during the treatment course of group A beta-haemolytic streptococcal pharyngitis. The isolated rheumatogenic strain M1 suggested the diagnosis of rheumatic fever. [ncbi.nlm.nih.gov]
Cardiomegaly is the most common radiological abnormality and ECG changes may show heart block but echocardiography is the most sensitive and shows pancarditis. Rheumatic Pancarditis - A gross photograph of the epicardial surface. [pedclerk.bsd.uchicago.edu]
[…] under the skin around the affected joints Unexplained weight loss Nervous system problems, such as involuntary movements and twitches (Sydenham’s chorea, also known as Saint Vitus’ dance) Heart problems such as inflammation (carditis), enlarged heart (cardiomegaly [betterhealth.vic.gov.au]
Plain radiograph pericardial calcification pulmonary edema pulmonary ossification : due to mitral valve disease 4 cardiomegaly global cardiomegaly from a dilated cardiomyopathy left atrial enlargement (particularly appendage) from mitral valve disease [radiopaedia.org]
Skin
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Subcutaneous Nodule
Subcutaneous nodule. The least common signs of ARF, these are found on the extensor surfaces of the extremities or along the spine. [web.archive.org]
According to this latest publication major manifestations are carditis, polyarthritis, chorea, erythema marginatum and subcutaneous nodules. [doi.org]
The frequency of subcutaneous nodules, invariably associated with carditis, was very high (33.3% in the first and 48.3% in recurrent episodes). Carditis was generally mild during first episode (53.3%) and severe with rheumatic recurrence (55.6%). [ncbi.nlm.nih.gov]
Clinical Description An inflammatory illness that occurs as a delayed sequela of group A streptococcal infection Major criteria : carditis, polyarthritis, chorea, subcutaneous nodules, and erythema marginatum Minor criteria : a) previous rheumatic fever [wwwn.cdc.gov]
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Skin Rash
The major criteria for diagnosis include: Arthritis in several large joints Heart inflammation Nodules under the skin Rapid, jerky movements (chorea, Sydenham chorea ) Skin rash The minor criteria include: Fever High ESR Joint pain Abnormal EKG You'll [web.archive.org]
The major criteria for diagnosis include: Arthritis in several large joints Heart inflammation Nodules under the skin Rapid, jerky movements (chorea, sydenham chorea ) Skin rash The minor criteria include: Fever High ESR Joint pain Abnormal EKG You'll [nlm.nih.gov]
Symptoms of rheumatic fever include sore or swollen joints (knees, elbows, ankles and wrists), a skin rash, a fever, stomach pain and/or jerky movements. [northlanddhb.org.nz]
Other symptoms include a red, blotchy, skin rash, which appears in 1 in 10 cases. Less common are nosebleeds, abdominal pain, bumps and lumps, or nodules, under the skin, and a high fever over 102 degrees Fahrenheit. [medicalnewstoday.com]
Some of its symptoms are tenderness and inflammation about the joints, fever, jerky movements, nodules under the skin, and skin rash. [infoplease.com]
Musculoskeletal
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Arthritis
Magnetic resonance imaging revealed suspected arthritis and osteomyelitis. The patient was treated for septic arthritis, but was subsequently diagnosed with ARF, after the development of carditis. [ncbi.nlm.nih.gov]
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Arthralgia
High ASO titers, arthralgia, and combination of high ASO titers with arthralgia are the main presenting symptoms in the group with a different diagnosis. [ncbi.nlm.nih.gov]
The minor criteria include- arthralgia, fever, heart block, leucocytosis, raised ESR or CRP and history of rheumatic fever. [lifehugger.com]
[…] inflammatory illness that occurs as a delayed sequela of group A streptococcal infection Major criteria : carditis, polyarthritis, chorea, subcutaneous nodules, and erythema marginatum Minor criteria : a) previous rheumatic fever or rheumatic heart disease; b) arthralgia [wwwn.cdc.gov]
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Migratory Polyarthritis
All cases presented with migratory polyarthritis, whereas 1 had erythema marginatum and transient carditis. These patients responded well to treatment with antibiotics and nonsteroidal antiinflammatory drugs. [ncbi.nlm.nih.gov]
Polyarthritis: Migratory polyarthritis is present in up to 75% of the cases and mainly involves large joints such as knees, ankles, elbows and wrists. [symptoma.com]
The classic history of joint involvement in acute rheumatic fever is one of large joint migratory polyarthritis. The inflammation lasts about two to three days in each joint and most often resolves without any sequelae. [news-medical.net]
polyarthritis pan carditis Sydenham chorea erythema marginatum subcutaneous nodules minor criteria fever arthralgia increase in acute phase reactants (e.g. [radiopaedia.org]
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Joint Swelling
Abdominal pain Fever Heart problems, which may not have symptoms, or may lead to shortness of breath and chest pain Joint pain, arthritis (mainly in the knees, elbows, ankles, and wrists) Joint swelling, redness, or warmth Nosebleeds Ring-shaped or snake-like [web.archive.org]
Acute rheumatic fever is often accompanied by joint swelling and pain ( arthritis ). Arthritis occurs in 75% of first attacks of rheumatic fever. [emedicinehealth.com]
Symptoms Symptoms of rheumatic fever may include: Fever Stomach pain Joint pain (arthritis) - primarily in the knees, elbows, ankles, and wrists Joint swelling Skin nodules Nosebleeds Rash on torso, arms, and legs that looks ring or snake-like Heart problems [heartdisease.about.com]
The most common symptoms of acute rheumatic fever are: Joint swelling and pain: This may include redness and warmth, mainly of the larger joints like the knees, ankles, wrists and elbows Fever: Hot and cold fevers which may feel like symptoms of a cold [rhdaustralia.org.au]
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Ankle Pain
Pain in one single joint that moves to another joint Red, hot, swollen joints Shortness of breath Fever and Perspiring Vomiting A flat, slightly raised, ragged rash Jerky, uncontrollable motions of their hands, feet, and face A decrease in an attention [healthlifemedia.com]
Common symptoms of rheumatic fever include: small, painless nodules under the skin chest pain rapid fluttering or pounding chest palpitations lethargy or fatigue nosebleeds stomach pain painful or sore joints in the wrists, elbows, knees, and ankles pain [healthline.com]
Face, Head & Neck
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Epistaxis
Clinical presentation Signs and symptoms of rheumatic fever include fever, migratory arthritis, abdominal pain, erythema marginatum (a ring-shaped rash located on trunk and upper parts of arms and legs), Sydenham's chorea, subcutaneous nodules, epistaxis [web.archive.org]
In some cases, epistaxis and abdominal pain may also be present. The diagnosis is made with the help of Duckett Jones criteria. The presence of two major criteria; or one major and two minor criteria is diagnostic. [symptoma.com]
Epistaxis: Epistaxis may be associated with severe protracted rheumatic carditis. Fever: Fevers greater than 39 C with no characteristic pattern are present initially in almost every patient with acute RF. [slideshare.net]
It is uncommon, reported in Other Clinical Manifestations Manifestations not included in the diagnostic criteria include epistaxis and abdominal pain, each of which occur in 5% of cases, and may occur before major manifestations. [clinicaladvisor.com]
Abdominal pain, rapid sleeping pulse rate, tachycardia out of proportion to fever, malaise, anemia, leukocytosis, epistaxis, and precordial pain also may be noted in patients with ARF. [dx.doi.org]
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Facial Grimacing
Descriptions have included facial grimaces, tongue movements ("bag of worms"), fidgetiness, halting and explosive speech, " pronator sign," " milkmaid's grip," " spooning," and clumsiness. [clinicaladvisor.com]
Psychiatrical
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Mood Swings
Sedatives and tranquilizers (such as diazepam or chlorpromazine) may be prescribed to treat Sydenham’s chorea, the unusual complication of rheumatic fever that causes jerky, uncontrolled movements and mood swings. [healthcommunities.com]
Neurologic
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Chorea
Sydenham's chorea, a major manifestation of rheumatic fever, was the most common cause of chorea in children in the past. But the incidence of Sydenham's chorea has declined in recent years in concert with the decline in rheumatic fever. [ncbi.nlm.nih.gov]
Chorea. Syndenham's chorea occurs in about 15% of patients with ARF and is more common in prepubertal girls. It is characterized by emotional labilty, personality changes, poor motor coordination and the classic purposeless spontaneous movements. [web.archive.org]
" JONES " J oints- Polyarthritis O (imagine 'O' as the Heart) - Carditis N odules (subcutaneous) E rythrema marginatum S ydenham' s chorea (St Vitus dance) Or, ACNES- Arthritis, Carditis, Nodules, Erythema marginatum and Sydenham' s chorea. [lifehugger.com]
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Lethargy
Typical symptoms of strep neck include: Small, painless nodules, or bumps, beneath the skin Upper body pain Rapid fluttering or chest that is pounding Fatigue or lethargy Nosebleeds Stomach pain Painful or joints that are sore the wrists, elbows, knees [healthlifemedia.com]
Common symptoms of rheumatic fever include: small, painless nodules under the skin chest pain rapid fluttering or pounding chest palpitations lethargy or fatigue nosebleeds stomach pain painful or sore joints in the wrists, elbows, knees, and ankles pain [healthline.com]
He presented to a local GP – accompanied by his grandmother – complaining of lethargy and pain in his knees and ankles. He had intermittent fever and his grandmother said he had been unwell for a 'long time'. [racgp.org.au]
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Tic Disorder
If this were correct, secondary prophylaxis that prevents recurrences of Sydenham chorea might also be effective in preventing recurrences of obsessive-compulsive and tic disorders in these patients. [doi.org]
disorder Reactive arthropathy Fibroelastoma Choreoathetoid cerebral palsy Lyme disease Congenital mitral valve disease Encephalitis Sickle cell anemia Congenital aortic valve disease Familial chorea (including Huntington disease) Infective endocarditis [dx.doi.org]
Workup
The diagnosis of rheumatic fever is usually clinical. The following investigations may be helpful.
- Throat swab culture: This is done to look for group A streptococci.
- Anti-streptolysin O titer (ASOT): This indicates a recent streptococcal infection and is raised in up to 80% of the cases [6] [7].
- Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP): These indicate active inflammation and are raised in the acute phase of the disease.
- Blood tests: Leukocytosis is seen with a total of around 12000 to 15000 white cells per cubic millimeter. Mild to moderate normocytic, normochromic anemia may be present.
- Chest X-ray: Chest X-ray will either be normal or will show signs of cardiomegaly, pulmonary edema and/or increased pulmonary vascularity.
- Electrocardiography (ECG): There may be prolonged PR interval, heart block or features of pericarditis and myocarditis.
- Echocardiography: Mitral regurgitation, myocardial dysfunction and pericardial effusion may be seen.
X-Ray
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Pericardial Effusion
However, serous pericardial effusion contained high levels of Epstein-Barr virus (EBV) DNA. Clonally proliferating EBV T cells were determined in the circulation. [ncbi.nlm.nih.gov]
Pericarditis may present with chest pain, friction rub, pericardial effusion and EKG changes. Signs of CHF may be present. Chorea. Syndenham's chorea occurs in about 15% of patients with ARF and is more common in prepubertal girls. [web.archive.org]
Echocardiography: Mitral regurgitation, myocardial dysfunction and pericardial effusion may be seen. The treatment of rheumatic fever has the following components. [symptoma.com]
Other abnormalities detected on echocardiography in acute carditis include prolapse of the valve, focal nodular thickening of leaflets and pericardial effusion. [doi.org]
PR Interval
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Prolonged PR Interval
We report on a 10-year-old boy who presented with progressive right side involuntary movements, an apical systolic murmur, prolonged PR interval, and elevated antistreptolysin O titer, who was diagnosed with acute rheumatic fever. [ncbi.nlm.nih.gov]
PR interval on an electrocardiogram Laboratory Criteria for Diagnosis No specific laboratory test exists for the diagnosis of rheumatic fever Case Classification Confirmed An illness characterized by a) two major criteria or one major and two minor criteria [wwwn.cdc.gov]
Electrocardiography (ECG): There may be prolonged PR interval, heart block or features of pericarditis and myocarditis. Echocardiography: Mitral regurgitation, myocardial dysfunction and pericardial effusion may be seen. [symptoma.com]
Blocks
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First-Degree Atrioventricular Block
First degree atrioventricular block is a common manifestation of acute rheumatic fever and is included in the Jones criteria but Wenckebacks phenomena and complete heart block are relatively rare manifestations of rheumatic fever. [ncbi.nlm.nih.gov]
Other ECG Findings
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Electrocardiogram Change
The minor criteria for diagnosis include fever, elevated ESR (erythrocyte sedimentation rate or CRP [ C-reactive protein ], nonspecific laboratory signs of inflammation), joint pain ( arthralgia ), EKG ( electrocardiogram ) changes, and history of previous [medicinenet.com]
Biopsy
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Aschoff Body
bodies in the myocardium and skin. 390 Rheumatic fever without mention of heart involvement convert 390 to ICD-10-CM Free ICD-9-CM Codes 2015 / Index · 2014 / Index · 2013 / Index 2012 / Index · 2011 / Index · 2010 / Index 2009 / Index · 2008 / Index [icd9data.com]
It is characterized by the formation of granulomatous lesions called Aschoff bodies usually in the heart tissue. [fpnotebook.com]
Postmortem histology 3 weeks after PET/CT showed Aschoff bodies with Anitschkow cells, pathognomonic for rheumatic carditis. [ncbi.nlm.nih.gov]
Treatment
The treatment of rheumatic fever has the following components [8] [9].
Bed rest: The patient should be advised complete bed rest until the temperature, resting pulse, erythrocyte sedimentation rate (ESR) and electrocardiogram (ECG) return to normal.
Aspirin: Aspirin and other salicylates are very effective in reducing the fever and relieving joint pain and swelling in the patients suffering from rheumatic fever. Aspirin therapy is continued for a period of 2 weeks. If the polyarthritis is controlled by then, the dosage is tapered for an additional 6 weeks. This is necessary because sudden discontinuation of aspirin therapy can cause recurrence of symptoms.
Corticosteroids: If the response to aspirin therapy is inadequate or if there is severe arthritis or carditis, a short course of steroids is given for 2 weeks after which the dosage is tapered over a period of 3 weeks.
Prognosis
During the initial phase, symptoms may last for several months in children and several weeks in adults. The mortality rate in this phase is 1 to 2%.
Valve disease occurs in up to two thirds of the patients by 10 years; however, not all the patients will develop symptoms or cardiomegaly.
Poor prognosis is implied if the patient develops persistent rheumatic carditis associated with cardiomegaly, heart failure or pericarditis.
Etiology
Rheumatic fever usually follows a pharyngeal infection caused by beta-hemolytic streptococcal species after a latent period of approximately 3 weeks. It results from the cross-reaction of the body’s immune response to the streptococcal antigen with its own tissues - principally those of the heart [1] [2].
Epidemiology
Rheumatic fever is much more prevalent in the developing countries where the incidence is as high as 1 case per 1000 population. In the developed countries, factors such as improved hygiene and living conditions, decreased crowding, the use of antibiotics and proper treatment have greatly reduced the incidence of rheumatic fever [3] [4].
Rheumatic fever commonly occurs in children aged 5 to 15 years - the peak age being 8 years. It is rare before the age of 4 and occasional cases are seen after the age of 30.
Pathophysiology
The acute phase of rheumatic fever is characterized by exudative and proliferative inflammatory reactions involving the heart, joints, brain, skin and subcutaneous tissues.
Rheumatic carditis principally involves the mitral (75-80%) and aortic (30%) valves. The valve cusps become thickened by edema and by infiltration of capillaries. Later on, a row of vegetations forms along the lines of closure of the valve leaflets. Inflammation of the valves leads to mitral and aortic regurgitation. In addition to valvulitis, there may be myocarditis and pericarditis.
Prevention
Primary prevention: The development of rheumatic fever can be stopped if there is prompt recognition and proper treatment for group A streptococcal pharyngitis and tonsillitis. Intramuscular benzathine penicillin is the antimicrobial agent of choice. In the patients allergic to penicillin, erythromycin or azithromycin may be used [10].
Secondary prevention: The recurrence of rheumatic fever can be prevented by continuous antimicrobial prophylaxis. An injection of benzathine penicillin should be given every 4 weeks. Salphasalazine or erythromycin are given in the patients who can not tolerate penicillin [11].
Summary
Rheumatic fever is an acute inflammatory disease that follows infection with group A beta-hemolytic streptococci. It is characterized by inflammatory lesions of connective tissue, mainly the heart, blood vessels and joints.
The patients may develop carditis, polyarthritis, Syndenham’s chorea, erythema marginatum and subcutaneous nodules. In addition, there may be fever, abdominal pain and other non-specific features.
Patient Information
Rheumatic fever occurs as a delayed sequela of throat infection with certain species of bacteria. It occurs much more commonly in children as compared to adults. The disease affects many parts of the body, in particular the heart and the joints. If the throat infection with the causal bacteria is detected and treated early, rheumatic fever can be prevented. The treatment of rheumatic fever is mostly symptomatic. Antibiotics are used to prevent the disease from occurring after it has been successfully controlled.
References
- Waksman BH. The etiology of rheumatic fever: a review of theories and evidence. 1949. Medicine. Jul 1993;72(4):262-272; discussion 278-283.
- Benderly A, Etzioni A. Role of the immune system in the etiology of rheumatic fever. Survey of immunologic research. 1985;4(4):319-324.
- Adanja B, Vlajinac H, Jarebinski M. Socioeconomic factors in the etiology of rheumatic fever. Journal of hygiene, epidemiology, microbiology, and immunology. 1988;32(3):329-335.
- Gordis L. The virtual disappearance of rheumatic fever in the United States: lessons in the rise and fall of disease. T. Duckett Jones memorial lecture. Circulation. Dec 1985;72(6):1155-1162.
- Shulman ST. T. Duckett Jones and his criteria for the diagnosis of acute rheumatic fever. Pediatric annals. Jan 1999;28(1):9-12.
- Machado CS, Ortiz K, Martins Ade L, Martins RS, Machado NC. [Antistreptolysin O titer profile in acute rheumatic fever diagnosis]. Jornal de pediatria. Mar-Apr 2001;77(2):105-111.
- Roy SB, Sturgis GP, Massell BF. Application of the antistreptolysin-O titer in the evaluation of joint pain and in the diagnosis of rheumatic fever. The New England journal of medicine. Jan 19 1956;254(3):95-102.
- Sociedade Brasileira de C. [Brazilian guidelines for the diagnosis, treatment and prevention of rheumatic fever]. Arquivos brasileiros de cardiologia. Sep 2009;93(3 Suppl 4):3-18.
- David L. [Diagnosis and treatment of rheumatic fever]. Archives de pediatrie : organe officiel de la Societe francaise de pediatrie. Jun 1998;5(6):681-686.
- Gerber MA, Baltimore RS, Eaton CB, et al. Prevention of rheumatic fever and diagnosis and treatment of acute Streptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics. Circulation. Mar 24 2009;119(11):1541-1551.
- Mispireta Dibarbout A, Chuquiure Lardizabal E, Corvacho de Campos A, Mispireta Vargas JL. [Active rheumatic fever. Diagnosis, treatment and prevention]. Boletin medico del Hospital Infantil de Mexico. Mar-Apr 1974;31(2):237-266.