Prof.Dr.Selma KARABEY

 Rheumatic fever is a common cause of acquired heart disease in children and adolescents living in poor socioeconomic conditions.  Acute rheumatic fever follows untreated or inadequately treated group A streptococcal infection of the tonsillopharynx and manifests after a latent period of about three weeks. (WHO,2011).

 Acute rheumatic fever primarily affects the heart, joints and central nervous system.  The major importance of acute rheumatic fever is its ability to cause fibrosis of heart valves leading to crippling haemodynamics of valvular heart disease, heart failure and death.  Surgery is often required to repair or replace heart valves in patients with severely damaged valves,  the cost of which is very high and a drain on the limited health resources of poor countries (WHO,2011).

 Rheumatic fever and rheumatic heart disease continue to exert a significant burden on the health of low socioeconomic populations in LMICs.  The disease almost disappeared in the developed world over the past century.  The decline of rheumatic fever in developed countries is believed to be the result of improved living conditions and availability of antibiotics for treatment of group A streptococcal infection.

 60% of acute rheumatic fever (ARF) cases would go on to develop rheumatic heart disease ( RHD) each year.  The remaining 40% of new ARF cases each year) with a history of ARF but no carditis presently requiring secondary prophylaxis.  Finally, there were over 492,000 deaths per year due to RHD, with approximately 468,000 of these occurring in less developed countries.

 ARF is the most prevalent cause of heart diseases in the year group  RHD is the most prevalent cause of cardiac diseases under 45 years old.  According to estimates,10-20 millions new cases occur each year.  Morbidity and sequelas are more important than mortality in ARF.  At least 15.6 million people are estimated to be currently affected by RHD.  RHD impacts children and young adults living in low-income countries. (WHO,2011)

 Rheumatic fever and rheumatic heart disease remain important public health problems in the world.  Rheumatic fever mostly affects children in developing countries where poverty, overcrowding, malnutrition, and inadequate medical care are found.  Globally, almost 2% of deaths from cardiovascular diseases is related to rheumatic heart disease, while 42% of deaths from cardiovascular diseases is related to ischaemic heart disease, and 34% to cerebrovascular disease.

“Rheumatic heart disease: A neglected heart disease of the poor”

 In 2005, it was estimated that over 2.4 million children aged 5-14 years are affected with RHD and 79% of all RHD cases come from less developed countries.  Further, the annual number of new ARF cases in children aged 5-14 years was more than 336,000.  95% of cases come from less developed countries.

 Even in industrialized societies, a relatively high prevalence of rheumatic fever persists in pockets of poverty, and outbreaks have been reported in affluent areas.  Despite that ARF is demonstrably preventable and RHD has declined dramatically in most industrialized nations, this condition remains a major public health problem.  The overall ratio of males to females is approximately 4:5, confirming that RHD is slightly more common in women.

Global Atlas on cardiovascular disease prevention and control, WHO 2013

Global Atlas on cardiovascular disease prevention and control, WHO 2013

Genetic predisposition:  Although the mechanism is not clear, genetic predisposition has been determined.  If family history is positive, the patient should be examined carefuly.  If there is a upper respiratory tract infection then throat culture must be done. If positive, anti-streptococcic therapy should be given.

First ARF attack  is seen between 5-15 years old.  is rare under 5 years old.  While ASO titration (serologic finding of streptoccoccal upper respiratory tract infection) is lower under 2 years old, is above the normal values between the 6-14 years old.

 During the 1960s, the incidence of acute rheumatic fever ranged from 23 to 55 per urban children aged 2-14 years in the United States.  In some areas of South America, the prevalence of the acute rheumatic fever is significantly higher, ranging from 1 to 10 per cent of scool-aged children (PAHO 1970)  Similar high rates are seen in areas of Asia and Africa. Reported prevalence in school children ranges from 1 to 78/1000 (WHO 2004)

 Turkish Rheumatic Fever Study Group declared that the prevalence of ARF was 10.8/ in the patients of pediatric outpatient departments.  Prevalence was 1.9 % in the patients of pediatric cardiology outpatient departments (14 centers, year of 2000)  According to a study carried out in Ankara, it has been determined that ARF prevalence has decreased 9-10 times during last 20 years (1995).

 The mechanisms by which this infection produces the clinical syndrome of acute rheumatic fever and subsequent rheumatic heart disease is well studied.(WHO 2004)  A group A streptococcal infection of throat ( tonsillopharyngitis) can be followed,in approximately 3 weeks, by an episode of acute rheumatic fever.  The rheumatic fever attack results in an inflammatuary reaction which involves the heart, joints and/or the central nervous system.

 Symptoms of rheumatic heart disease include: shortness of breath, fatigue, irregular heart beats, chest pain and fainting.  Symptoms of rheumatic fever include: fever, pain and swelling of the joints, nausea, stomach cramps and vomiting.

A firm diagnosis requires that  two major or  one major and two minor criteria are satisfied in addition to evidence of recent streptococcal infection.

 Carditis  Polyarthritis (knees, ankles, elbows and wrists)  Chorea (Syndenham’s chorea/St. Vitus´ dance)  Erythema Marginatum  Subcutaneous nodules

 Fever  Arthralgia  Previous rheumatic fever or rheumatic heart disease  Acute phase reactants (Leukocytosis, elevated ESR and CRP)  Prolonged P-R interval on electrocardiogram

Any one of the following is considered adequate evidence of infection:  Increased antistreptolysin O or other streptococcal antibodies  Positive throat culture for Group A beta- hemolytic streptococci  Positive rapid direct Group A strep carbohydrate antigen test  Recent scarlet fever.

 Rapid antigen tests for the diagnosis of group A streptococcal throat infections are highly specific, but less sensitive.  While a positive test suggests the need for treatment, a negative test indicates the need for throat culture.(Dajani et al. 1995)  Antibody tests can confirm a recent group A streptococcal infection.

“Poverty alleviation and better living conditions are key for prevention of rheumatic heart disease” (WHO, 2011)

 Primary prevention is achieved by treatment of acute throat infections caused by group A streptococcus. This effect may be achieved at relatively low cost if a single intramuscular penicillin injection is administered.  Secondary prevention is used following an attack of acute rheumatic fever to prevent the progression to cardiac disease and has to be continued for many years.  Secondary prevention programmes are currently thought to be more cost effective for prevention of rheumatic heart disease than primary prevention and may be the only feasible option for LMICs in addition to poverty alleviation efforts.

 Primary prevention of acute rheumatic fever is the recommended aproach  Throat cultures should be performed on all patients with tonsillopharangitis and those with a positive culture for group A streptococcal infections treated (Dajani et al. 1950)  Antibiotic treatment can effectively prevent acute rheumatic fever even when given up to 9 days from the onset of the infection(Denny et al. 1950)  Antibiotic treatment can be either oral or by injection

 Primary prophylaxis is a proven method of prevention, however has not to date been proven to be cost-effective, resulting in secondary prophylaxis remaining the mainstay of RF/RHD management, as do IM benzathine benzylpenicillin, oral phenoxymethylpenicillin and oral erythromycin.

 Early treatment of streptococcal sore throat can stop the development of rheumatic fever.  Regular long-term penicillin treatment can prevent repeat attacks of rheumatic fever which give rise to rheumatic heart disease and can stop disease progression in people whose heart valves are already damaged by the disease.

 Firstly whether IM benzathine benzylpenicillin (considered first line for secondary prophylaxis) should be administered every four weeks, versus every two or three weeks.  The internationally accepted dose for the secondary prevention of ARF in adults is 1.2 million IU.  Current pharmacokinetic evidence suggests 600,000 IU be given to patients weighing less than 20kg, and 1.2 million IU be given to all other patients.

 The individuals with a history of acute rheumatic fever, the likelihood of secondary attacks with additional damage is common, estimated to be approximately 50 per cent of those with an antibiotic is recommended (Dajani et al. 1995)  If group A streptococcal infections are appropriately detected and treated, rheumatic heart disease can be effectively prevented.  In those where it is not prevented, lifelong valvular heart disease results in diminishing function and premature mortality.

Duration of prophylaxis for:  Arthritis and Chorea : until 20 years old,  Carditis: lifelong “benzathine benzylpenicillin” In order to stop prophylaxis:  Recurrency should not have been seen in last 5 years.

 Oxford Textbook of Public Health,Cardiovasculer and Cerebrovasculer Diseases,Roger Detels, Robert Beaglehole, Mary Ann Lansang, Martin Gulliford, Oxford Univercity Press, 2009  Maxcy-Rosenau-Last Public Health and Preventive Medicine, Heart Disease, Robert B.Wallace, Neal Kohatsu, 2007  Global Atlas on Cardiovascular disease prevention and control,Shanti Mentis, Pekka Puska, Bo Norrving,WHO in Collaboration with the World Heart Federation and the World Stroke Organization, 2011  The community control of rheumatic fever and rhuumatic heart disease:report of a WHO international cooperative project, Bulletin of the World Health Organization, 59(2) , T.Strasser,N.Dondog, A.El Kholy, 1981  Cardiovasculer diseases(CVDs) WHO   Jones Criteria   Treatment of rheumatic fever  medicines/committees/subcommittee/2/RheumaticFever-review.pdf medicines/committees/subcommittee/2/RheumaticFever-review.pdf  TTB, STED,’’On soru on yanıt’’, Volume 12, number 2,2003 