Rheumatic fever is an inflammatory disease which may develop after a Group A streptococcal infection (such as strep throat or scarlet fever) and can involve the heart, joints, skin, and brain. It commonaly appears in children ages 5 through 15 . The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // I00-I99 - Diseases of the circulatory system (I00-I02) Acute rheumatic fever (I00) Rheumatic fever without mention of heart involvement (I01) Rheumatic fever with heart involvement (I02) Rheumatic chorea (I05-I09) Chronic rheumatic heart diseases (I05) Rheumatic mitral valve diseases (I050) Mitral stenosis (I051) Rheumatic mitral insufficiency (I06) Rheumatic aortic... // I00-I99 - Diseases of the circulatory system (I00-I02) Acute rheumatic fever (I00) Rheumatic fever without mention of heart involvement (I01) Rheumatic fever with heart involvement (I02) Rheumatic chorea (I05-I09) Chronic rheumatic heart diseases (I05) Rheumatic mitral valve diseases (I050) Mitral stenosis (I051) Rheumatic mitral insufficiency (I06) Rheumatic aortic... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... An abscess on the skin, showing the redness and swelling characteristic of inflammation. ... This article is about the medical term. ... The group A streptococcus bacterium (Streptococcus pyogenes, or GAS) is a form of Streptococcus bacteria responsible for most cases of streptococcal illness. ... Strep throat (or Streptococcal pharyngitis, or Streptococcal Sore Throat) is a form of Group A streptococcal infection that affects the pharynx. ... The heart and lungs, from an older edition of Grays Anatomy. ... For other uses, see Joint (disambiguation). ... Beyond overall skin structure, refer below to: See-also. ... The human brain In animals, the brain (enkephalos) (Greek for in the skull), is the control center of the central nervous system, responsible for behavior. ...

General information

Rheumatic fever is common worldwide and responsible for many cases of damaged heart valves. In the Western countries, it became fairly rare since the 1960s, probably due to widespread use of antibiotics to treat streptococcus infections. While it is far less common in the United States since the beginning of the 20th century, there have been a few outbreaks since the 1980s. Although the disease seldom occurs, it is serious and has a mortality of 2–5%. Grays Fig. ... Streptococcus is a genus of spherical shaped Gram-positive bacteria, belonging to the phylum Firmicutes[1] and the lactic acid bacteria group. ... (19th century - 20th century - 21st century - more centuries) Decades: 1900s 1910s 1920s 1930s 1940s 1950s 1960s 1970s 1980s 1990s As a means of recording the passage of time, the 20th century was that century which lasted from 1901–2000 in the sense of the Gregorian calendar (1900–1999... The 1980s refers to the years from 1980 to 1989. ...

Rheumatic fever primarily affects children between ages 6 and 15 years and occurs approximately 20 days after strep throat or scarlet fever. In up to a third of cases, the underlying strep infection may not have caused any symptoms.

The rate of development of rheumatic fever in individuals with untreated strep infection is estimated to be 3%. The rate of development is far lower in individuals who have received antibiotic treatment. Persons who have suffered a case of rheumatic fever have a tendency to develop flare-ups with repeated strep infections.

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. Heart complications may be long-term and severe, particularly if valves are involved.

Diagnosis: modified Jones criteria

T. Duckett Jones, MD, first published these criteria in 1944.^[1] They have been periodically revised by the American Heart Association in collaboration with other groups.^[2] Two major criteria, or one major and two minor criteria, when there is also evidence of a previous strep infection, support the diagnosis of rheumatic fever.^[3][4] The American Heart Association (AHA) is a non-profit organization in the United States that fosters appropriate cardiac care in an effort to reduce disability and deaths caused by cardiovascular disease and stroke American Stroke Association Web site. ...

Major criteria

• Carditis: inflammation of the heart muscle which can manifest as congestive heart failure with shortness of breath, pericarditis with a rub, or a new heart murmur.
• Migratory polyarthritis: a temporary migrating inflammation of the large joints, usually starting in the legs and migrating upwards.
• Sydenham's chorea (St. Vitus' dance): a characteristic series of rapid movements without purpose of the face and arms. This can occur very late in the disease.
• Erythema marginatum: a long lasting rash that begins on the trunk or arms as macules and spread outward to form a snakelike ring while clearing in the middle. This rash never starts on the face and is made worse with heat.
• Subcutaneous nodules (a form of Aschoff bodies): 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.

Congestive heart failure (CHF), also called congestive cardiac failure (CCF) or just heart failure, is a condition that can result from any structural or functional cardiac disorder that impairs the ability of the heart to fill with or pump a sufficient amount of blood throughout the body. ... Pericarditis is inflammation of the sac surrounding the heart, the pericardium. ... Murmurs are abnormal heart sounds that are produced as a result of turbulent blood flow, which is sufficient to produce audible noise. ... Arthritis (from Greek arthro-, joint + -itis, inflammation; plural: arthritides) is a group of conditions where there is damage caused to the joints of the body. ... Sydenhams chorea (or Rheumatic chorea) is a disease characterized by rapid, uncoordinated jerking movements affecting primarily the face, feet and hands. ... Erythema marginatum is described as the presence of pink rings on the trunk and inner surfaces of the arms and legs which come and go for as long as several months. ... The macule is the simplest dermatological lesion. ... Aschoff bodies or Aschoff nodules are painless nodules present in rheumatic fever. ...

Minor criteria

• Fever: temperature elevation
• Arthralgia: Joint pain without swelling
• Laboratory abnormalities: increased Erythrocyte sedimentation rate, increased C reactive protein, leukocytosis
• Electrocardiogram abnormalities: a prolonged PR interval
• Evidence of Group A Strep infection: positive culture for Group A Strep, elevated or rising Antistreptolysin O titre
• Previous rheumatic fever or inactive heart disease

This article needs additional references or sources for verification. ... This article does not cite any references or sources. ... The erythrocyte sedimentation rate (ESR), also called a sedimentation rate, sed rate or Biernacki Reaction, is a non-specific measure of inflammation that is commonly used as a medical screening test. ... C-reactive protein (CRP) is a plasma protein, an acute phase protein produced by the liver. ... Leukocytosis is an elevation of the white blood cell count (the leukocyte count) above the normal range. ... “QRS” redirects here. ... Antistreptolysin O titre (ASO titre or ASOT) - titre of (serum) antistreptolysin O antibodies; a blood test used to assist in the diagnosis of a streptococcal infection or indicate a past exposure to streptococci. ...

Other signs and symptoms

• Abdominal pain
• Nosebleeds

Abdominal pain can be one of the symptoms associated with transient disorders or serious disease. ... A nosebleed or nosebleedage, medically known as epistaxis, is the relatively common occurrence of hemorrhage (bleeding) from the nose, usually noticed when it drains out through the nostrils. ...

Pathophysiology

Rheumatic fever is a systemic disease affecting the peri-arteriolar connective tissue and can occur after an untreated Group A streptococcal pharyngeal infection. It is believed to be caused by antibody cross-reactivity. This cross-reactivity is a Type II hypersensitivity reaction and is termed molecular mimicry. Usually, self reactive B cells remain anergic in the periphery without T cell co-stimulation. During a Strep. infection activated antigen presenting cells such as macrophages present the bacterial antigen to helper T cells. Helper T cells subsequently activate B cells and induce the production of antibodies against the cell wall of Streptococcus. However the antibodies may also react against the myocardium and joints^[5], producing the symptoms of Rheumatic fever. Cross-reactivity is the reaction between an antigen and an antibody that was generated against a different but similar antigen. ...

Group A streptococcus pyogenes has a cell wall composed of branched polymers which sometimes contain "M proteins" that are highly antigenic. The antibodies which the immune system generates against the "M proteins" may cross react with cardiac myofiber sarcolemma and smooth muscle cells of arteries, inducing cytokine release and tissue destruction. This inflammation occurs through direct attachment of complement and Fc receptor-mediated recruitment of neutrophils and macrophages. Characteristic Aschoff bodies, composed of swollen eosinophilic collagen surrounded by lymphocytes and macrophages can be seen on light microscopy. The larger macrophages may become Aschoff giant cells. Acute rheumatic valvular lesions may also involve a cell-mediated immunity reaction as these lesions predominantly contain T-helper cells and macrophages.^[6] Binomial name Streptococcus pyogenes Rosenbach 1884 Streptococcus pyogenes is a Gram-positive coccus that grows in long chains depending on the culture method. ... Plant cells separated by transparent cell walls. ... A polymer is a long, repeating chain of atoms, formed through the linkage of many molecules called monomers. ... An antigen is a molecule that stimulates the production of antibodies. ... Muscle system The sarcolemma is the cell membrane of a muscle cell. ... Cytokines are a group of proteins and peptides that are used in organisms as signaling compounds. ... Cell-mediated immunity is an immune response that does not involve antibodies but rather involves the activation of macrophages and NK-cells, the production of antigen-specific cytotoxic T-lymphocytes, and the release of various cytokines in response to an antigen. ... This article or section does not adequately cite its references or sources. ... Macrophages (Greek: big eaters) are cells found in tissues that are responsible for phagocytosis of pathogens, dead cells and cellular debris. ...

In acute RF, these lesions can be found in any layer of the heart and is hence called pancarditis. The inflammation may cause a serofibrinous pericardial exudates described as “bread-and-butter” pericarditis, which usually resolves without sequelae. Involvement of the endocardium typically results in fibrinoid necrosis and verrucae formation along the lines of closure of the left-sided heart valves. Warty projections arise from the deposition, while subendothelial lesions may induce irregular thickenings called MacCallum plaques.

Chronic rheumatic heart disease is characterized by repeated inflammation with fibrinous resolution. The cardinal anatomic changes of the valve include leaflet thickening, commissural fusion and shortening and thickening of the tendinous cords. RHD cause 99% of mitral stenosis often resulting in a “fish mouth” gross appearance.^[7]

Treatment

The management of acute rheumatic fever is geared 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. Another important cornerstone in treating rheumatic fever includes the continuous use of low dose antibiotics (such as penicillin, sulfadiazine, or erythromycin) to prevent recurrence. Anti-inflammatory refers to the property of a substance or treatment that reduces inflammation. ... This article is about the drug. ... In physiology, corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. ... Staphylococcus aureus - Antibiotics test plate. ... For the Japanese rock band, see Penicillin (band). ... Sulfadiazine is a sulphonamide. ... Erythromycin is a macrolide antibiotic which has an antimicrobial spectrum similar to or slightly wider than that of penicillin, and is often used for people who have an allergy to penicillins. ...

Infection

Patients with positive cultures for streptococcus pyogenes should be treated with penicillin as long as allergy is not present. This treatment will not alter the course of the acute disease. Allergy is an abnormal reaction to a substance foreign to the body that is acquired, predictable and rapid. ...

Inflammation

Patients with significant symptoms may require corticosteroids. Salicylates are useful for pain. In physiology, corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. ... Salicylic acid is a colorless, crystalline organic carboxylic acid. ...

Heart failure

Some patients develop significant carditis which manifests as congestive heart failure. This requires the usual treatment for heart failure: diuretics and digoxin. Unlike normal heart failure, rheumatic heart failure responds well to corticosteroids. Carditis is the inflammation of the heart or its surroundings. ... Congestive heart failure (CHF), also called congestive cardiac failure (CCF) or just heart failure, is a condition that can result from any structural or functional cardiac disorder that impairs the ability of the heart to fill with or pump a sufficient amount of blood throughout the body. ... A diuretic is any drug that tends to increase the flow of urine from the body (diuresis). ... Digoxin (INN) (IPA: ) is a purified cardiac glycoside extracted from the foxglove plant, Digitalis lanata. ... In physiology, corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. ...

Prevention

References

JAMA, published continuously since in 1883, is an international peer-reviewed general medical journal published 48 times per year. ... Year 2007 (MMVII) is the current year, a common year starting on Monday of the Gregorian calendar and the AD/CE era in the 21st century. ... is the 195th day of the year (196th in leap years) in the Gregorian calendar. ... Year 2007 (MMVII) is the current year, a common year starting on Monday of the Gregorian calendar and the AD/CE era in the 21st century. ... is the 195th day of the year (196th in leap years) in the Gregorian calendar. ...

External links

• Rheumatic fever information from Seattle Children's Hospital Heart Center