Rheumatic fever Classification and external resources
ICD-10	I00.-I02.
ICD-9	390–392
DiseasesDB	11487
MedlinePlus	003940
eMedicine	med/3435  med/2922 emerg/509 ped/2006
MeSH	D012213

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. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The International Statistical Classification of Diseases and Related Health Problems 10th Revision ( ICD -10) is a coding of diseases and signs symptoms abnormal findings I00-I99 - Diseases of the Circulatory system (I00-I02 Acute rheumatic fever ( Rheumatic fever without mention of Heart I00-I99 - Diseases of the Circulatory system (I00-I02 Acute rheumatic fever ( Rheumatic fever without mention of Heart The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. The Diseases Database is a free Website that provides information about the relationships between medical conditions Symptoms, and Medications. MedlinePlus, with the MedlinePlus Medical Encyclopedia, is a website network containing Health information from the world's largest medical Library eMedicine is an online clinical medical knowledge base that was founded in 1996 by Scott Plantz and Richard Lavely two medical doctors Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books Inflammation ( Latin, inflamatio, to set on fire is the complex biological response of vascular tissues to harmful stimuli such as Pathogens A disease is an abnormal condition of an organism that impairs bodily functions and can be deadly The group A streptococcus bacterium ( Streptococcus pyogenes, or GAS) is a form of Streptococcus bacteria responsible for most cases Streptococcal pharyngitis or streptococcal sore throat ( Strep throat AmE) is a form of Group A streptococcal infection that affects the Scarlet fever is a disease caused by an Exotoxin released by Streptococcus pyogenes. The heart is a muscular organ in all Vertebrates responsible for pumping Blood through the Blood vessels by repeated rhythmic A joint is the location at which two or more Bones make contact The skin is the outer covering of living tissue of an animal (or plant The brain is the center of the Nervous system in animals All Vertebrates and the majority of Invertebrates have a brain It commonly appears in children ages 6 through 15 .

Contents 1 General information 2 Diagnosis: modified Jones criteria 2.1 Major criteria 2.2 Minor criteria 2.3 Other signs and symptoms 3 Pathophysiology 4 Treatment 4.1 Infection 4.2 Inflammation 4.3 Heart failure 5 Prevention 6 Notable people affected or killed by rheumatic fever 7 References 8 External links

General information

Rheumatic fever is common worldwide and responsible for many cases of damaged heart valves. In Anatomy, the heart valves are Valves in the Heart that maintain the unidirectional flow of blood by opening and closing depending on the difference In the Western countries, it became fairly rare since the 1960s, probably due to widespread use of antibiotics to treat streptococcus infections. Streptococcus is a Genus of spherical Gram-positive bacteria belonging to the phylum Firmicutes and the 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. The United States of America —commonly referred to as the The twentieth century of the Common Era began on The 1980s was the decade spanning from January 1 1980 to December 31 1989. Although the disease seldom occurs, it is serious and has a mortality of 2–5%. ^[1]

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 incidence of recurrence with a subsequent untreated infection is substantially greater (about 50%). ^[2] 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. ^[3] They have been periodically revised by the American Heart Association in collaboration with other groups. The American Heart Association (AHA is a Non-profit organization in the United States that fosters appropriate ^[4] 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. Exceptions are chorea and indolent carditis, each of which by itself can indicate rheumatic fever. Chorea sancti viti (Latin for " St Vitus' dance " is an abnormal involuntary movement disorder one of a group of neurological disorders called Dyskinesias ^[5][6][7]

Major criteria

The mnemonic JONES is often used to recall the Major Criteria. A mnemonic device (nəˈmɒnɪk is a Memory aid Commonly met mnemonics are often verbal something such as a very short poem or a special word used to help a person remember

• Joints (Migratory polyarthritis): a temporary migrating inflammation of the large joints, usually starting in the legs and migrating upwards. Arthritis (from Greek arthro-, joint + -itis, inflammation plural arthritides is a group of conditions involving damage to the Joints of the body
• O [imagine heart-shaped O] (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. Carditis is the inflammation of the Heart or its surroundings Heart failure is a Cardiac condition that occurs when a problem with the structure or function of the Heart impairs its ability to supply Pericarditis is an Inflammation ( -itis) of the Pericardium (the fibrous sac surrounding the heart Murmurs are abnormal Heart sounds that are produced as a result of turbulent blood flow which is sufficient to produce audible noise
• Nodules (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. Aschoff bodies or Aschoff nodules are painless nodules present in Rheumatic fever. These now occur infrequently.
• Erythema marginatum: a long lasting rash that begins on the trunk or arms as macules and spreads outward to form a snakelike ring while clearing in the middle. Erythema is redness of the Skin caused by Capillary congestion 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. It is flat and can only be seen and not felt This rash never starts on the face and is made worse with heat.
• Sydenham's chorea (St. Sydenham's chorea (also known as " Saint Vitus Dance " is a disease characterized by rapid uncoordinated jerking movements affecting primarily the face feet and hands Vitus' dance): a characteristic series of rapid movements without purpose of the face and arms. This can occur very late in the disease.

an additional way to remember the major criteria is by the mnemonic: C. A. N. C. ER

• C: Carditis
• A: Arthritis
• N: Nodules (sub cutaneous)
• C: Chorea
• ER: ERythema Marginatum

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

Other signs and symptoms

• Abdominal pain
• Nosebleeds

Pathophysiology

Rheumatic fever is a systemic disease affecting the peri-arteriolar connective tissue and can occur after an untreated Group A streptococcal pharyngeal infection. Fever (also known as pyrexia, from the Greek pyretos meaning fire or a febrile response, from the Latin word Febris Arthralgia (from Greek arthro-, joint + -algos, pain literally means joint pain; it is a symptom of injury infection illnesses -- in particular The erythrocyte sedimentation rate (ESR also called a sedimentation rate, sed rate, or Biernacki Reaction, is the rate at which Red blood cells C-reactive protein ( CRP) is a Plasma protein, an Acute phase protein produced by the Liver and by Adipocytes. Leukocytosis is a raised white blood cell count (the Leukocyte count above the normal range Antistreptolysin O titre ( AS(LO titre or AS(LOT) - Titre of ( serum) antistreptolysin O Antibodies is a Blood test Abdominal pain can be one of the Symptoms associated with transient disorders or serious disease Epistaxis (or a nosebleed in Plain English) is the relatively common occurrence of Hemorrhage from the Nose, usually noticed when the blood drains It is believed to be caused by antibody cross-reactivity. Cross-reactivity is the reaction between an Antigen and an Antibody that was generated against a different but similar antigen 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^[8], producing the symptoms of rheumatic fever.

Group A streptococcus pyogenes has a cell wall composed of branched polymers which sometimes contain "M proteins" that are highly antigenic. Streptococcus pyogenes is a spherical Gram-positive Bacteria that grows in long chains and is the cause of Group A streptococcal infections A cell wall is a tough flexible and sometimes fairly rigid layer surrounding a cell, located external to the Cell membrane, which provides the cell with structural A polymer is a large Molecule ( Macromolecule) composed of repeating Structural units typically connected by Covalent Chemical bonds An antigen (from antibody-generating) or immunogen is a substance that prompts the generation of Antibodies and can cause an immune response The antibodies which the immune system generates against the "M proteins" may cross react with cardiac myofiber protein myosin^[9] and smooth muscle cells of arteries, inducing cytokine release and tissue destruction. Myosins are a large family of Motor proteins found in Eukaryotic tissues. Cytokines are a category of signalling Proteins and Glycoproteins that like Hormones and Neurotransmitters, are used extensively in cellular 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. Cell-mediated immunity is an Immune response that does not involve Antibodies or complement but rather involves the activation of Macrophages T helper cells (also known as effector T cells or Th cells) are a sub-group of Lymphocytes (a type of White blood cell or Macrophages ( Greek: "big eaters" from makros "large" + phagein "eat" ( Mø) are cells within the tissues that ^[10]

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. Pericarditis is an Inflammation ( -itis) of the Pericardium (the fibrous sac surrounding the heart 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. ^[11]

Treatment

The management of acute rheumatic fever is geared toward the reduction of inflammation with anti-inflammatory medications such as aspirin or corticosteroids. Anti-inflammatory refers to the property of a substance or treatment that reduces Inflammation. Aspirin, or acetylsalicylic acid (ASA (əˌsɛtɨlsælɨˌsɪlɨk ˈæsɨd is a Salicylate drug, often used as an Analgesic to relieve Corticosteroids are a class of Steroid hormones that are produced in the Adrenal cortex. Individuals with positive cultures for strep throat should also be treated with antibiotics. In modern usage an antibiotic is a Chemotherapeutic agent with activity against Microorganisms such as Bacteria, fungi or Protozoa Aspirin is the drug of choice and should be given at high doses of 100mg/kg/day. One should watch for side effects like gastritis, salicylate poisoning etc. Steroids are reserved for cases where there is evidence of involvement of heart. The use of steroids may prevent further scarring of tissue and may prevent development of sequelae such as Mitral stenosis. Monthly injections of Longacting Penicillin must be given for a period of 5 years in patients having one attack of Rheumatic fever. If there is evidence of carditis, the length of Penidure 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. Penicillin (sometimes abbreviated PCN or pen) is a group of Beta-lactam antibiotics used in the treatment of Bacterial Infections Sulfadiazine is a sulfonamide Antibiotic. It eliminates bacteria that cause infections by stopping the production of Folic acid inside the bacterial cell Erythromycin is a Macrolide Antibiotic that has an antimicrobial spectrum similar to or slightly wider than that of Penicillin, and is often used for people

Infection

Patients with positive cultures for streptococcus pyogenes should be treated with penicillin as long as allergy is not present. Allergy is a disorder of the Immune system often also referred to as Atopy. This treatment will not alter the course of the acute disease.

Inflammation

Patients with significant symptoms may require corticosteroids. Corticosteroids are a class of Steroid hormones that are produced in the Adrenal cortex. Salicylates are useful for pain. Salicylic acid (from the Latin word for the willow tree Salix, from whose bark it can be obtained is a Beta hydroxy acid (BHA with the formula

Heart failure

Some patients develop significant carditis which manifests as congestive heart failure. Carditis is the inflammation of the Heart or its surroundings Heart failure is a Cardiac condition that occurs when a problem with the structure or function of the Heart impairs its ability to supply This requires the usual treatment for heart failure: diuretics and digoxin. A diuretic is any Drug that elevates the rate of urination ( Diuresis) Digoxin ( INN) (dɨˈdʒɒksɨn also known as Digitalis, is a purified Cardiac glycoside extracted from the Foxglove plant Digitalis Unlike normal heart failure, rheumatic heart failure responds well to corticosteroids. Corticosteroids are a class of Steroid hormones that are produced in the Adrenal cortex.

Prevention

Prevention of recurrence is achieved by eradicating the acute infection and prophylaxis with antibiotics. Prophylaxis ( Greek "προφυλάσσω" to guard or prevent beforehand) is any medical or Public health procedure whose purpose The American Heart Association recommends daily or monthly prophylaxis continue long-term, perhaps for life. The American Heart Association (AHA is a Non-profit organization in the United States that fosters appropriate ^[12]

Nurses also have a role in prevention, primarily in screening school-aged children for sore throats that may be caused by Group A streptococci(especially Group A β Hemolytic Streptococcus pyogenes).

Notable people affected or killed by rheumatic fever

• Bobby Bowden
• Jeremy Brett
• Lou Costello
• Bobby Darin
• Kurt Gödel
• Franz Kline
• Carson McCullers
• Wolfgang Amadeus Mozart (likely though not certain)
• Richard Proenneke

References

External links

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

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