Rheumatic fever 412735 224337267 2008-07-08T12:07:19Z Graham87 194203 /* Notable people affected or killed by rheumatic fever */ rm Grace Farris, doesn't have a Wikipedia article {{Infobox_Disease | Name = Rheumatic fever | Image = | Caption = | DiseasesDB = 11487 | ICD10 = {{ICD10|I|00||i|00}}-{{ICD10|I|02||i|00}} | ICD9 = {{ICD9|390}}–{{ICD9|392}} | ICDO = | OMIM = | MedlinePlus = 003940 | eMedicineSubj = med | eMedicineTopic = 3435 | eMedicine_mult = {{eMedicine2|med|2922}} {{eMedicine2|emerg|509}} {{eMedicine2|ped|2006}} | MeshID = D012213 | }} '''Rheumatic fever''' is an [[autoimmune]] [[inflammatory disease|inflammatory]] [[disease]] which may develop after a [[Group A streptococcal infection]] (such as [[strep throat]] or [[scarlet fever]]) and can involve the [[heart]], [[joint]]s, [[skin]], and [[brain]]. It commonly appears in children ages 6 through 15 .{{Fact|date=February 2008}} ==General information== Rheumatic fever is common worldwide and responsible for many cases of damaged [[heart valve]]s. 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%.<ref>[http://www.nlm.nih.gov/medlineplus/ency/article/003940.htm|NLM/NIH: Medline Plus Medical Encyclopedia: Rheumatic fever]</ref> 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%).<ref>Porth, C. Essentials of pathophysiology: Concepts of altered health states. 2nd Edition. Lippincott, Williams & Wilkins. 2007.</ref> 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.<ref>Jones TD. The diagnosis of rheumatic fever. ''[[Journal of the American Medical Association|JAMA]]''. 1944; 126:481–484</ref> They have been periodically revised by the [[American Heart Association]] in collaboration with other groups.<ref>[http://circ.ahajournals.org/cgi/content/full/106/19/2521?ck=nck Ferrieri P. Proceedings of the Jones criteria workshop]. ''Circulation'' 2002; 106: 2521–23</ref> '''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 (disease)|chorea]] and [[indolent carditis]], each of which by itself can indicate rheumatic fever. <ref>{{cite web |url=http://www.emedicine.com/emerg/topic509.htm |title=eMedicine — Rheumatic Fever | author = Steven J Parrillo, DO, FACOEP, FACEP |accessdate=2007-07-14 |format= |work=}}</ref><ref>{{cite journal |author= |title=Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association |journal=JAMA |volume=268 |issue=15 |pages=2069–73 |year=1992 |pmid=1404745 |doi=}}</ref><ref>{{cite journal|first=Anita|last=Saxena|title=Diagnosis of rheumatic fever: Current status of Jones criteria and role of echocardiography|journal=Indian Journal of Pediatrics|volume=67|issue=4|pages=283–286|year=2000|pmid=11129913|doi=10.1007/BF02758174}}</ref> ===Major criteria=== The [[mnemonic]] '''JONES''' is often used to recall the Major Criteria. *'''J'''oints ([[Arthritis|Migratory polyarthritis]]): a temporary migrating inflammation of the large joints, usually starting in the legs and migrating upwards. *'''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]]. *'''N'''odules (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. *[[Erythema|'''E'''rythema]] marginatum: a [[Erythema marginatum|long lasting rash]] that begins on the trunk or arms as [[macule]]s and spreads outward to form a snakelike ring while clearing in the middle. This rash never starts on the face and is made worse with heat. *[[Sydenham's chorea|'''S'''ydenham'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. 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]] *[[Nosebleed]]s ==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<ref>Abbas and Lechtman. '''Basic Immunology: Functions and Disorders of the Immune System'''. Elsevier Inc. 2004.</ref>, 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]]. The antibodies which the immune system generates against the "''M proteins''" may cross react with cardiac myofiber protein [[myosin]]<ref>K. C. Fae, D. D. da Silva, S. E. Oshiro et al. "Mimicry in Recognition of Cardiac Myosin Peptides by Heart-Intralesional T Cell Clones from Rheumatic Heart Disease" Journal of Immunology, May 1, 2006; 176(9): 5662 - 5670. http://www.jimmunol.org/cgi/content/abstract/176/9/5662 </ref> 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]].<ref>Kumar et al. '''Robbins and Cotran Pathologic Basis of Disease'''. Elsevier Inc. 2005</ref> 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. <ref>{{cite web |url=http://www.robbinspathology.com/ |title=Robbins & Cotran Pathologic Basis of Disease |accessdate=2007-07-14 |format= |work=}}</ref> ==Treatment== The management of acute rheumatic fever is geared toward the reduction of inflammation with [[anti-inflammatory medication]]s such as [[aspirin]] or [[corticosteroid]]s. Individuals with positive cultures for strep throat should also be treated with [[antibiotic]]s. 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. ===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. ===Inflammation=== Patients with significant symptoms may require [[corticosteroids]]. [[Salicylates]] are useful for pain. ===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]]. ==Prevention== Prevention of recurrence is achieved by eradicating the acute infection and [[prophylaxis]] with antibiotics. The [[American Heart Association]] recommends daily or monthly prophylaxis continue long-term, perhaps for life.<ref name="aha">{{cite web|title=Rheumatic Heart Disease/Rheumatic Fever|url=http://www.americanheart.org/presenter.jhtml?identifier=4709|publisher=American Heart Association|accessdate=2008-02-17}}</ref> 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]] *[[Leleiohoku II|William Pitt Leleiohoku]] *[[Carson McCullers]] *[[Wolfgang Amadeus Mozart]] (likely though not certain) *[[Charles Stewart Parnell]] (likely though not certain) *[[Richard Proenneke]] ==References== {{Reflist|2}} ==External links== * [http://heartcenter.seattlechildrens.org/conditions_treated/rheumatic_heart_disease.asp Rheumatic fever information] from Seattle Children's Hospital Heart Center {{Circulatory system pathology}} [[Category:Cardiology]] [[Category:Bacterial diseases]] [[Category:Rheumatology]] [[ar:حمى روماتيزمية]] [[zh-min-nan:Hong-sip-jia̍t]] [[de:Rheumatisches Fieber]] [[es:Fiebre reumática]] [[fr:Rhumatisme articulaire aigu]] [[it:Febbre reumatica]] [[nl:Acuut reuma]] [[ja:リウマチ熱]] [[pl:Gorączka reumatyczna]] [[pt:Febre reumática]] [[sr:Реуматска грозница]] [[fi:Reumakuume]] [[sv:Reumatisk feber]]