Acute Rheumatic Fever PDF

Acute rheumatic fever is a delayed non- suppurative sequela of streptococcal throat infection The most common cause of acquired heart disease in childhood in developing countries  It is a common diesease with high incidence and prevelance,  I may cause mortality or disability  It h...

Acute rheumatic fever is a delayed non- suppurative sequela of streptococcal throat infection The most common cause of acquired heart disease in childhood in developing countries  It is a common diesease with high incidence and prevelance,  I may cause mortality or disability  It has a high cost to the community  is a diffuse inflamatory disease of connective tissue  involving ▪ heart, ▪ joints, ▪ brain, ▪ blood vessels ▪ subcutaneous tissue.  ARF follows streptococcal throat infection but it is not seen after the skin infections.  3-6 % of any population is susceptible to acute rheumatic fever  Most important risk factor is poverty  The incidence in industrialized countries is below 5/100000. (The incidence of rheumatic heart disease in school children in Japan and USA is 0.6-0.7/100000)  In middle east and mediterranean countries 25- 100/ 100000.  In underdevelopd countries more than 100/100000.  There are 15 600 000 people with rheumatic heart disease  500 000 new cases are seen with acute rheumatic fever  300 000 new cases develop rheumatic heart disease  233 000 deaths occur due to rheumatic heart disease or acute rheumatic fever “ Rheumatic fever is a disease that licks the joints but bites the heart”  Occurs weeks after a streptococcal throat infection Acute Rheumatic fever: (usually 2-4 weeks). It is an autoimmune reaction where the body’s - first attack immune system attacks its own tissues, mistakenly - reactivation (recurrence) recognizing them as similar to streptococcal antigens eg: arthritis (molecular mimicry). carditis chorea Chronic Rheumatic Heart Disease: eg: mitral insufficiency What ar tbe scute , chronic sings of aortic insufficency rhemoatic heart diseas? mitral stenosis ► Gram(+) ► Prevelant in nature ► Arranged in chains when cultured  Acording to their ability to hemolyse RBC (, , )  Acording to a polysaccharide antigen in the carbohydrate layer of the cell wall (A, B, C, D...) Group A- Streptococcus pyogenes Responsible for %90 of human streptococcal infections  M types- (>150) Cell membrane protein, Virulance factor,Antigenic Nephritogenic (12, 49) Rheumatogenic (1, 3, 5, 6, 14, 18, 19, 24, 27, 29)  Streptococcal component Mammalian tissue constituent  Streptococcal hyaluronic acid Mammalian hyaluronic acid protein polysaccharide  Group A carbohydrate Glycoproteins of heart valves  Protein cell wall Sarcolemma of cardiac & skeletal muscle  Protein cell membrane Sarcolemma of cardiac & skeletal muscle  Glycoprotein of cell membrane Glycoprotein of glomeruler basement membrane  Antigen of cell membrane Histocompatibility antigen  Hemolysins (Streptolysin O) Antistreptolysin  Streptokinase Antistreptokinase  Hyaluronidase Anti hyaluronidase  Deoxyribonucleases ( A,B,C,D) Anti DNB  Streptococcal esterase Anti streptococcal esterase  The prevelance of group A streptococci in throat cultures of healthy school children is between 10 to 50 %.  Elevated ASO titers may be found in 15 to 69% of healthy school children in various studies.  Attack rate of ARF following a streptoccoccal pharingitis is 0.3 to 3 %.  Host factors:  Age between 5 to 15 years  Genetic susseptibility  No difference in gender, race or ethnic group  HLA B5  Environmental factors  Late winter, early spring  Crowded household  Socioeconomically deprivated populations  Under developed or developing countries Patogenesis of Rheumatic Fever: vulnerable human host streptococcal infection genetic predisposition upper respiratory tract age special strain season toxic strep products socio-economic factors cross-reactive immunity (strep antigens and human heart antigens) immune complex disturbed helper and/or suppressor T cells ACUTE RHEUMATIC FEVER  Exudative  Proliferative (Aschoff bodies)  1-5 weeks  Longer in Chorea ( up to 6 months) Carditis Poliarthritis Chorea Erythema marginatum Subcutaneous nodules  Which on ethe belwo is right about Arthritis?  %70-80  Large joint involvement (knee, ankle, elbow, wrist)  Acute arthritis lasting less than a few weeks You will see a complete heailing of Arthritis in ARF.  More than one joint is involved  Migratory nature  Swelling, heat, tenderness, limitation of motion  Dramatic response to salycilates  No sequela  %50  Pancarditis Endocardium Myocardium Pericardium  If carditis is present during the initial attack, it is likely during the following attacks  Valvular regurgitations during the acute phase, late development of stenosis  Murmurs due to valvular regurgitation  Mitral valve and aortic valve are commonly involved  Mitral regurgitation- Apical pansistolic murmur Carey-Coombs murmur  Aortic regurgitation- Parasternal diastolic murmur Austin-Flint Murmur  Tricuspid and pulmonary valve involvement is rare   Sinus tachycardia  Cardiomegaly on chest x-ray  Congestive heart failure  Rhythm and conduction abnormalities what would you expect to see in myocardial involment what are the signs of pericardial involvment /  Chest pain  Friction rub  Distant heart sounds  Pericardial effusion  ECG changes  Some children with carditis may have a very insidious onset disease, without any cardiac signs during physical examination. They have general disease symptoms such as fever, fatigue, anorexia or paleness. Echocardiography reveals severe cardiac involvement. The latent period is long in these patients and presence of carditis is sufficient for diagnosis. WHAT KIND OF CARDIC ENVOLVMETN WOULD YOU EXPWCT TO SEE IN SIENT CARDITIS  In patients with arthritis or chorea as the major I silent carditis patient actually do not have crditis. Ture manifestations, and without clinical carditis; echocardiography may reveal mild valvular regurgitations. This is called as silent carditis.  In recent (2015) update of diagnostic criteria (Jones Criteria) silent cardits is considered as a major manifestation as well as clinical carditis.  %15-20  More common in prepubertal girls  Personality changes, emotional lability  Purposeless, involuntary, jerky movements  Motor weakness  Disturbed hand writing, difficulty in buttoning  Self limited (3 months)  Long latent period  Incidious carditis is common  %10  More common in patients with carditis  Trunk and proximal portions of the extremities  Serpiginious macular erythematous lesions with pale center and pink borders  Nonpruritic  %5-10  0.2-2cm in diameter  Hard, mobile, painless,non-pruritic swellings  Extensor surfaces of extremities  Sculp  Spine  More common in patients with carditis Clinical findings - Arthralgia (Must not be considered in the presence of arthritis) Fever (>38C) Laboratory findings- Elevated acute phase reactants ESR (> 60 mmHg) CRP (Not affected by anemia or endocarditis) Prolonged PR interval (Must not be considered in the presence of carditis) For low risk populations ( Incidence of ARF< 2/100000, prevelance of RHD38.5°C) Laboratory findings - Elevated acute phase reactants (ESR > 60mm/hr, CRP>3mg/dl) Prolonged PR interval For moderate/high risk populations ( Incidence of ARF> 2/100000, prevelance of RHD >1/1000)  MAJOR MANIFESTATIONS Carditis (Clinical/echocardiographic diagnosis) Arthritis (Poliarthritis, monoarthritis or poliarthralgia) Chorea Erythema marginatum Subcutaneous nodules  MINOR MANIFESTATIONS Clinical findings - Monoarthralgia Fever (>38°C) Laboratory findings - Elevated acute phase reactants (ESR > 30mm/hr, CRP3>mg/dl) Prolonged PR interval SUPPORTING EVIDENCE OF ANTECEDENT GROUP A STREPTOCOCCAL INFECTION  Pozitive throat culture or rapid streptococcal antigen test  Elevated or rising streptococcal antibody titer (ASO, Anti DNAsB)  History of scarlet fever (for high risk populations)  2 major criteria  1 major+2 minor criteria  3minör criteria  if supported by evidence of recent streptococcal infection diagnosis is possible  Nose bleeding  Abdominal pain  Rheumatic pneumonia  Perfect without cardiac involvement  Valvular heart disease in patients with carditis  Recurrenses worsens the prognosis  Recurrent attacks mostly in the first five years  Juvenile rheumatoid arthritis  Immune complex disease  Other arthropathies (septic arthritis,toxic synovitis)  Infective endocarditis  Henoch-Schoenlein purpura  Sickle cell anemia  Acute leukemia  Systemic lupus erithematosus  Viral myocarditis  Functional murmurs  Mitral valve prolapse  Poliomyelitis  Acute apendicitis  Primary prophylaxis is the treatment of upper respiratory tract infection due to group A streptococci to prevent an initial attack of acute rheumatic fever.  Secondary prophylaxis is the regular administration of penicillin to a patient who has had rheumatic fever in order to prevent colonization or infection of upper respiratory tract with group A streptococci and subsequent development of recurrences.  Benzathine penicillin 600000 u im. for < 27kg 1200000u im. for > 27 kg  Phenoxymethyl penicillin/Amoxicilline 3x 250 mg oral for children 3x500 mg oral for adults (10 days)  Cephalexin 50mg/kg/day :2 (10 days)  Erythromycine 30 mg / kg / day:2 (10 days)  Benzathine Penicillin 1200000u im once in every 3 weeks  Phenoxymethyl penicillin 2x 500mg / day or 2x 1000mg/day oral  Erythromycine 2x250mg /day oral  Azithromycin 5mg/kg/day (max 250mg) ► Patients without carditis should recieve prophylaxis for a minimum of 5 years after the last attack and at least until the age 21. ► Patients with carditis during acute attack but without cardiac sequela may recive secondary prophylaxis for 10 years or until 25 years of age. ► Patients with carditis and rheumatic heart disease should continue prophylaxis for lifelong. ► Patients with chorea should recieve prophylaxis until age 21, if carditis is not present.  Secondary prophylaxis for rheumatic fever should not be confused with antibiotic prophylaxis used for prevention of infective endocarditis (Patients with rheumatic heart disease should recieve both)  Patients who had undergone cardiovascular surgery are still under the risk of recurrences and require prophylaxis.  Initial laboratory tests (throat culture, ASO, chest x-ray, ECG, acute phase reactants-ESR,CRP)  Eradication of streptococci with administration of Benzathine penicillin  Initiation of secondary prophylaxis  Bed-chair rest  Arthritis  4-6 weeks  Carditis  6-12 weeks ( Avoid physical activities for 3 months ) Immunosuppressive treatment  Acetylsalicylic acid 75-100mg/kg/day (max 3gr) for patients with arthritis lasting 4- 6 weeks  Corticosteroids: Prednisolone 2mg/kg/day (max 60mg) for patients with carditis lasting 6-12 weeks  Salycilates  Naproxen  Ibuprofene Corticosteroids and salycilates suppress clinical symptoms and acute phase rectants but does not shorten the duration of rheumatic activity.  Sedatives Haloperidole Valproic acid  Prevention of accidents  Digoxin  Diuretics  ACE inhibitors  Valvulotomi  Valvuloplasty  Valve replacement