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Rheumatic Fever

Acute Rheumatic Fever

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:

The minor criteria include the following:

Cough
  • 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]
  • 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]
  • Strep throat is infectious and can spread through cough and sneeze droplets.[spasifikmag.com]
Dyspnea
  • 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]
Fever
  • A greater clinical and laboratory vigilance is required for the diagnosis of acute rheumatic fever in the asymptomatic identical twin of a patient with rheumatic fever.[ncbi.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]
  • […] to make the diagnosis of acute rheumatic fever.[ncbi.nlm.nih.gov]
  • Six patients were known cases of rheumatic fever but had stopped penicillin prophylaxis. Commonest complaint in patients with rheumatic fever was joint pain followed by fever.[ncbi.nlm.nih.gov]
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]
  • […] your child's neuromuscular movements (this is usually noted by a change in your child's handwriting and may also include jerky movements) Rash (a pink rash with odd edges that is usually seen on the trunk of the body or arms and legs) Fever Weight loss Fatigue[chw.org]
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]
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.edu]
  • 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]
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]
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]
  • 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]
  • pain; nosebleeds; weakness; and loss of appetite and body weight.[britannica.com]
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]
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]
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]
  • Rheumatic heart disease point prevalence was 3.2 cases per 1,000 children in August 2013.[ncbi.nlm.nih.gov]
  • Cardiac involvement during acute rheumatic fever can result in rheumatic heart disease, which can cause heart failure and premature mortality.[ncbi.nlm.nih.gov]
  • A total of 20 333 children who were free of rheumatic heart disease at enrolment were available for analysis. During a mean follow-up time of 5 years, 96 children developed acute rheumatic fever or chronic rheumatic heart disease.[ncbi.nlm.nih.gov]
  • Recurrent acute rheumatic fever and rheumatic heart disease can be prevented by antibiotic intervention.[ncbi.nlm.nih.gov]
Chest Pain
  • Thus, patients presenting with chest pain and electrocardiological and biochemical evidence of myocardial necrosis are not always suffering from an acute coronary syndrome.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
Heart Murmur
  • murmur and/or acute chorea.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
Palpitations
  • 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]
  • People with RHD may have no symptoms for years or only mild symptoms (such as shortness of breath with exercise or on lying down, tiredness, weakness or palpitations) until complications develop.[sahealth.sa.gov.au]
  • In other cases, the person may have a range of symptoms, including breathlessness, chest pain and heart palpitations. Many Aboriginal and Torres Strait Islander children who have rheumatic fever are not diagnosed or treated.[betterhealth.vic.gov.au]
  • 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[healthline.com]
Tachycardia
  • The junctional tachycardia and other electrocardiogram abnormalities resolved during follow-up.[ncbi.nlm.nih.gov]
  • Other electrocardiographic changes such as sinus tachycardia, bundle branch blocks. nonspecific ST-T wave changes, atrial and ventricular premature complexes have been reported with variable frequency.[ncbi.nlm.nih.gov]
  • 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]
  • […] 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), rapid heartbeat (tachycardia[betterhealth.vic.gov.au]
  • Tachycardia is usual, although some children develop bradycardia. ST elevation suggests pericarditis. CXR may show features of heart failure.[patient.info]
Subcutaneous Nodule
  • 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]
  • After several weeks, multiple subcutaneous nodules as well as migratory polyarthritis or monoarthritis developed in both children, fulfilling Jones criteria of RF.[ncbi.nlm.nih.gov]
  • Joint pain was the commonest present complaint (29 patients), followed by fever in 17 patients, 32 had carditis, 26 had arthritis, 13 had subcutaneous nodules, 7 had chorea and one had erythema marginatum.[ncbi.nlm.nih.gov]
  • Major manifestations in the acute phase included carditis, 27 cases (61.4%); polyarthritis, 22 cases (50%); erythema marginatum, 7 cases (15.9%); Sydenham chorea, 3 cases (6.8%); and subcutaneous nodules, 1 case (2.3%).[ncbi.nlm.nih.gov]
  • Subcutaneous nodules: Small, hard, painless nodules may form under the skin.[symptoma.com]
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]
  • Monoarticular arthritis in children is most often suppurative septic arthritis (SA) of bacterial origin. We recently cared for 3 patients with monoarticular arthritis who developed carditis while receiving antibiotics for SA.[ncbi.nlm.nih.gov]
  • Six (28.6%) patients had monoarthritis, and 4 (19%) patients had migratory arthritis. No patient had carditis.[ncbi.nlm.nih.gov]
  • In this case, the administration of supraphysiologic doses of glucocorticoids caused iatrogenic Cushing syndrome with decreased bone mineral density and bilateral avascular tibial necrosis mimicking the symptoms of arthritis.[ncbi.nlm.nih.gov]
  • Carditis was present in all patients, arthritis in 37 % and Sydenham chorea in 32 %.[ncbi.nlm.nih.gov]
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]
  • 'Atypical' pattern was present in 63% (39/62) of patients with articular manifestations, of which arthralgia was a common manifestation (57%).[ncbi.nlm.nih.gov]
  • . • Our paper highlights the importance of including ARF in the differential diagnosis of a febrile child with arthritis/arthralgia and/or heart murmur and/or acute chorea.[ncbi.nlm.nih.gov]
  • Arthralgias: Patients may report arthralgias upon presentation.[slideshare.net]
  • […] prior rheumatic fever increased ESR prolonged PR interval (First degree heart block) prior Group A Strep infection Treatment Nonoperative oral antibiotics and anti-inflammatories indications acute infection confirmed by Modified Jones criteria arthralgias[orthobullets.com]
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]
  • After several weeks, multiple subcutaneous nodules as well as migratory polyarthritis or monoarthritis developed in both children, fulfilling Jones criteria of RF.[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]
Joint Swelling
  • 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]
  • Acute rheumatic fever is often accompanied by joint swelling and pain ( arthritis ). Arthritis occurs in 75% of first attacks of rheumatic fever.[emedicinehealth.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]
  • For those with symptoms, the main symptom is joint pains or full-blown arthritis (joint swelling and limited joint movement).[jamaicaobserver.com]
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[healthline.com]
Epistaxis
  • 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]
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]
Emotional Lability
  • It may include emotional lability, personality changes, muscular weakness, and uncoordinated, involuntary, purposeless movements.[childrensheartinstitute.org]
  • Chorea can be easily recognized, and a neurologist or rheumatologist is immediately contacted for medical care, since the embarrassing involuntary movements, the emotional lability, and the obsessive-compulsive disorders associated with the condition[scielo.br]
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]
  • An eight-year-old girl is presented with three major criteria of acute rheumatic fever: polyarthritis, carditis and chorea.[ncbi.nlm.nih.gov]
  • Carditis was present in all patients, arthritis in 37 % and Sydenham chorea in 32 %.[ncbi.nlm.nih.gov]
  • 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]
  • Three newly described cases, and three previous reports of Henoch-Schönlein purpura associated with rheumatic carditis or chorea were identified.[ncbi.nlm.nih.gov]
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[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]
Tic Disorder
  • PANDAS is a term used to describe certain children whose symptoms of neuropsychiatric conditions, such as obsessive-compulsive disorder or tic disorders, are worsened by strep infection.[mayoclinic.org]

Workup

The diagnosis of rheumatic fever is usually clinical. The following investigations may be helpful.

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]
  • Echocardiography: Mitral regurgitation, myocardial dysfunction and pericardial effusion may be seen. The treatment of rheumatic fever has the following components.[symptoma.com]
  • effusion, but an isolated pericardial effusion in the absence of valvular regurgitation is unlikely to be due to RF.[clinicaladvisor.com]
  • effusion myocardial inflammation Promoted articles (advertising)[radiopaedia.org]
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]
  • Initial attack patients have higher incidence of prolonged PR-interval (67 vs. 12%, P 0.049) and longer duration of admission to diagnosis (5 vs. 2 days, P 0.05). Thirty percent presented initial attack after 30 years of age.[ncbi.nlm.nih.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]
  • PR interval) Imaging Radiographs may show effusion but often unremarkable Ultrasound/MRI equivocal cases where concern remains for septic arthritis Echocardiogram Lab Studies Serum labs antistreptolysin O titers elevated in 80% peripheral blood leukocytosis[orthobullets.com]
  • 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]
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]
Aschoff Body
  • Postmortem histology 3 weeks after PET/CT showed Aschoff bodies with Anitschkow cells, pathognomonic for rheumatic carditis.[ncbi.nlm.nih.gov]
  • It is characterized by the formation of granulomatous lesions called Aschoff bodies usually in the heart tissue.[fpnotebook.com]
  • Microscopic appearance Anitschkow cells (caterpillar cells) may be seen which are macrophages with chromatin condensation that can fuse to become Aschoff bodies.[radiopaedia.org]
  • Sucutaneous nodules (a form of Aschoff bodies) is a painless, firm collections of collagen fibers on the back of the wrist, the outside elbow, and the front of the knees. These now occur infrequently.[newworldencyclopedia.org]

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.

Sex distribution
Age distribution

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

Article

  1. Waksman BH. The etiology of rheumatic fever: a review of theories and evidence. 1949. Medicine. Jul 1993;72(4):262-272; discussion 278-283.
  2. Benderly A, Etzioni A. Role of the immune system in the etiology of rheumatic fever. Survey of immunologic research. 1985;4(4):319-324.
  3. 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.
  4. 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.
  5. Shulman ST. T. Duckett Jones and his criteria for the diagnosis of acute rheumatic fever. Pediatric annals. Jan 1999;28(1):9-12.
  6. 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.
  7. 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.
  8. 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.
  9. 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.
  10. 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.
  11. 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.

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Last updated: 2018-06-22 09:47