Uploaded byDr. Renesha Islam
Acute Rheumatic Fever.ppt
This document provides an overview of acute rheumatic fever presented by Dr. Renesha Islam. Some key points: - Acute rheumatic fever is an immune response to Group A streptococcus infection that causes inflammation of the heart, joints, brain and skin. It often damages the heart valves long term. - Major symptoms include migratory polyarthritis in joints (75% of cases), carditis (50-60% of cases, involving the heart valves, myocardium and pericardium), Sydenham's chorea (10-15% of cases), erythema marginatum and subcutaneous nodules (rare). - Diagnosis is based on the revised Jones criteria
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The seminar on Acute Rheumatic Fever is led by Dr. Renesha Islam, specializing in Pediatric Hematology and Oncology.
Acute rheumatic fever is a post-infectious immune response to group A streptococcus, affecting joints and heart.
Rheumatic fever leads to chronic heart damage but is preventable; named for similarity to Rheumatism.
Caused primarily by Group A beta hemolytic streptococci (GABS), with specific serotypes more common.
High incidence in developing countries exceeds 50/100,000 children; peaks in ages 5-15 with varying demographic factors.
Two theories: Immunologic and Cytotoxicity; immunity and toxins from GAS may lead to ARF pathology.
Presence of Aschoff bodies in heart, joints, and subcutaneous tissues; affecting mainly mitral/aortic valves.
Jones Criteria for diagnosing initial and recurrent ARF attacks based on major/minor manifestations.Common features include migratory polyarthritis, carditis, chorea, erythema marginatum, and subcutaneous nodules.
ARF can be confused with conditions like rheumatoid arthritis, myocarditis and chorea.
Diagnosis through throat cultures, antibody titers, inflammatory markers, X-rays, ECG, and echocardiography.
Includes counseling, antibiotics for streptococcal infection, anti-inflammatory treatments, specific management for complications.
ARF resolves arthritis and chorea but may lead to significant heart diseases such as rheumatic valvular heart disease.
Two components for prevention: socioeconomic improvements and both primary and secondary antibiotic prophylaxis.
Prognosis depends on initial clinical manifestations and severity; recurrences increase risk of permanent heart damage.
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Acute Rheumatic Fever.ppt
- 1. Presenter: DR. RENESHA ISLAM RESIDENT(PHASE-A) PAEDIATRIC HEMATOLOGY & ONCOLOGY WELCOME TO SEMINAR ON ACUTE RHEUMATIC FEVER PRESENTED BY DR. RENESHA ISLAM Resident-Phase A (PAEDIATRIC HEMATOLOGY & ONCOLOGY)
- 2. ACUTE RHEUMATIC FEVER • Acuterheumatic fever is a post infectious immune response to group A streptococcus (GAS) that results in a diffuse inflammatory disease involving the joints and heart and less frequently the brain, skin and subcutaneous tissue. • It has a tendency to recur.
- 3. • Rheumatic fevercauses chronic progressive damage to the heart and its valves. • But it is the most preventable disease of all CVS diseases. • It is so named because of its similarity in presentation to Rheumatism.
- 4. Etiology Group Abeta hemolytic streptococci (GABS) Serotypes (M types 1,3,5,6,18,29) are more frequently isolated.
- 5. EPIDEMIOLOGY • The incidenceof ARF in some developing countries exceeds 50 per 1,00,000 children. • World wide , rheumatic heart disease remains the most common form of acquired heart disease in all age groups .
- 6. Epidemiology ( Cont…) •Incidence peaks in 5-15 yrs. age group. • More male patients of RF than female. • Females are more vulnerable to develop congestive heart failure than male. • Most of the patient belongs to the low socioeconomic group. • Higher incidence of streptococcal sore throat observed during winter.
- 7. EPIDEMIOLOGY • In theUSA at the beginning of the 20th century annual incidence was 100-200/1,00,000 population. • By the early 1980s the annual incidence was decreased as low as 0.5/1,00,000 population. • This Sharp decline in the incidence of ARF has been observed in other industrialized countries as well.
- 8. Several theories ofthe pathogenesis of acute rheumtic fever have been proposed. Main two are: a. IMMUNOLOGIC THEORY b. CYTOTOXICITY THEORY PATHOGENESIS
- 9. IMMUNOLOGIC THEORY • Ithas been suggested that patients of rheumatic fever produce antibodies against streptococcal cell wall and cell membrane proteins . • The streptococcal antigen and human myocardium appear to be identical antigenically.
- 10. IMMUNOLOGIC THEORY • Mostimportant antigenic proteins of GAS in external layer of cell wall are M ( M1 , M5 , M6 & M9 ) T, & R proteins. The following antigens of streptococci cross react to connective tissue antigens : • Streptococcal M protein cross react to cardiac myosin • Streptococcal membrane antigen cross reactive with myocardial smooth muscle sarcolemma. • Hyaluronate capsule of streptococcus cross-reactive with heart valve glycoprotein.
- 11. IMMUNOLOGIC THEORY • Otherantigenic Components – Cell wall group A carbohydrate, Capsular hyaluronate, protoplast membrane. • Immunogenic cross reactivity occurs between streptococcal components ( M protein , cell wall group A carbohydrate etc ) and tissues of heart , joints and brain .
- 13. IMMUNOLOGIC THEORY Cont. Stepsor sequence in pathogenesis : Streptococcus pyogenes-sore throat pharyngitis Antigen of S. Pyogenes Immune response (B cell or humoral) in 2-4 weeks. Antistreptococcal antibodies against the antigens. Antibodies react with cross-reactive antigens of conncetive tissue (heart, joints) Systemic inflammation of connective tissue.
- 14. CYTOTOXICITY THEORY • Thecytotoxicity theory suggests that a GAS toxin may be involved in the pathogenesis of acute rheumatic fever and rheumatic heart disease. • GAS produces several enzymes that are cytotoxic to mammalian cardiac cells , such as streptolysin O. • one of the major problems with the cytotoxicity hypothesis is its inability to explain the latent period between GAS pharyngitis and the onset of acute rheumatic fever.
- 15. PATHOLOGY Microscopically-Focal inflammatory noduleknown as Aschoff body which is pathognomonic of rheumatic fever. The nodule is formed by fibrinoid necrosis of collagen surrounded by lymphocytes, macrophage & occasional plasma cells. It may be found in all 3 layers of heart, joints & subcutaneous tissue. Macroscopically-Most obvious in the heart valves & the pericardium. The valve cusps become thickened by oedema & by the infiltration of capillaries.
- 16. PATHOLOGY Cont. These arecommon on the mitral or aortic valve cusp & seldom affect the tricuspid or pulmonary valves. When the pericardium is involved it becomes thickened, may contain a fibrinous straw coloured effusion.
- 17. Clinical Manifestations As becauseno clinical or laboratory finding is pathognomic for acute rheumatic fever, T. Duckett Jones in 1944 proposed guidelines to aid in dignosis and to limit over diagnosis. The Jones criteria , as revised in 2015 by AHA are for the diagnosis of the initial attack of ARF & recurrent attacks.
- 18. Guidelines for thediagnosis of initial attack of Rheumatic fever (Jones Criteria, Updated 2015) Major Manifestations Minor Manifestations Supporting Evidence of antecedent GAS infection. -Carditis -Polyarthritis -Erythema marginatum -Subcutaneous nodules -Chorea Clinical features: - Arthralgia - Fever Laboratory features: ●Elevated acute phase reactants - ESR - CRP ●Prolonged P-R interval - positive throat swab culture or - Rapid Streptococcal antigen test or - Elevated or increasing streptococcal antibody titer . The presence of 2 major or of 1 major and 2 minor manifestations indicates a high probability of ARF if supported by evidence of preceding GAS infection_ INITIAL ATTACK.
- 19. RECURRENT ATTACK – 2major or 1 major & 2 minor or 3 minor menifestations (the latter 1 only in the moderate/ high risk population plus evidence of recent GAS infection.
- 20. Exceptions: • There are3 circumstances in which the diagnosis of ARF can be made without strict adherence to the Jones criteria : 1.Chorea 2.Indolent carditis 3.Recurrences of acute RF, may or may not fulfill Jones criteria.
- 21. Migratory Polyarthritis - Mostcommon feature: present in 75% cases. - Typically involves larger Joints. (Knees, ankles, elbows, wrists) Involvement of spine, hips and small joints of the feet and hand are uncommon. - Joints are generally hot, red, swollen and exquisitely tender, even the friction of bedclothes is uncomfortable. Pain is more marked than swelling.
- 22. Migratory Polyarthritis (continued)…… - characteristically migratory in nature. - A dramatic response to salicylates (aspirin) is characteristic. - typically not deforming. If left untreated the joint swelling disappear spontaneously in 3-4 weeks time leaving no permanent deformities of joints.
- 23. • There isoften an inverse relationship between : the severity of arthritis and the severity of cardiac involvement .
- 24. Carditis -Most serious manifestation -Present in 50-60% cases. - Characteristic pancarditis (involves all the 3 layers of heart . i.e. endocardium, myocardium, pericardium)
- 25. Carditis (continued) …… -Endocardits (valvulitis) : is a universal finding in rheumatic Carditis. where as the presence of pericarditis or myocarditis is variable. - Murmurs present due to valvular involvement.
- 26. Carditis (continued) …… Mitralregurgitation is characterized by a high- pitched apical holosystolic murmur radiating to the axilla. There may be associated with an apical mid-diastolic murmur of relative mitral stenosis. Aortic insufficiency is characterized by a high-pitched decrescendo diastolic murmur at the upper left sternal border.
- 27. Carditis (continued) …… •Valvular insufficiency is characteristic of both acute & convalescent stages of ARF whereas valvular stenosis usually appears several years or even decades after the acute illness. • Most cases consist of either isolated mitral valvular disease or combined aortic and mitral valvular disease. Isolated aortic or right-sided valvular involvement is uncommon. • Serious and long-term illness is related entirely to valvular heart disease as a consequence of a single attack or recurrent attacks of acute rheumatic fever. -
- 28. Carditis (continued) …… Myocarditis: -Present with tachycardia( which is disproportionate to the degree of fever & is present during sleep), conduction defect, cardiomegaly. Gallop rhythm is common physical findings. Pericarditis: - Present with (chest pain ,Pericardial rub & effusion)
- 29. Sydenham’s Chorea - Occursin 10-15% cases. -Usually presents as an Isolated, frequently subtle , neurologic behavior disorder. - there is involuntary, purposeless, nonrepeatative movements of the limbs , face and trunk. .
- 30. -Clinical maneuvers toelicit features of chorea include : 1. Milk maid’s grip. (Irregular contractions of the muscles of the hands while squeezing the examiner's finger). 2. Spooning and pronation of hands when arms extended. 3. Wormian darting movements of tongue upon protrusion. 4. Deterioration of hand writing. (to evaluate fine motor movement)
- 31. - Exacerbated bystress disappeared with sleep. - Associated features : Emotional liability , incoordination , poor school performance , uncontrollable movements and facial grimacing.
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- 33.
- 34. Erythema Marginatum: - Itis rare , occurs in < 3% of patient but highly characteristic for ARF . - Erythematous, non-pruritic ,serpiginous , macular lesion with pale centres. -It occurs primarily on the trunk and extremities, but not on the face. -It can be accentuated by warming the skin .
- 35.
- 36. Subcutaneous Nodules: -these arerare (≤ 1% of patients) - Firm, non tender, freely movable nodules . Approx. 1 cm in diameter. -present along extensor surfaces of tendons near bony prominences. - There is a correlation between the presence of these nodules and carditis or significant RHD.
- 37. OTHER MANIFESTATIONS • Fever:remittent in nature, >102⁰ F • Arthralgia.
- 38. Differential Diagnoses Arthritis CarditisChorea Rheumatoid Arthritis Reactive arthritis Serum Sickness Sickle cell disease Malignancy SLE V. Myocarditis V. Pericarditis Infective endocarditis Kawasaki disease Congenital Heart Disease Mitral valve prolapse Innocent murmur Huntington chorea Wilson disease SLE Cerebral palsy Tics
- 39. Investigations Evidence ofrecent group A streptococcal infection- • Throat swab culture • Streptococcal antibodies titre Antistreptolysin O (ASO) Anti deoxyribonuclease (Anti DNase B) Antihyaluronidase (AH). If only single antibody is measured (ASO titer) only 80-85% of patients have an elevated titer. However , 95- 100 % have an elevation, if 3 different antibodies are measured.
- 40. Investigations contd… Evidenceof the systemic inflammation- » WBC » ESR - raised, CRP – raised. Evidence of Cardiac involvement- • Chest X-ray - Cardiomegaly, Pulmonary congestion. • ECG- prolonged P-R interval, tachycardia, T-wave inversion in pericarditis. • Echocardiography - Can provide a more accurate diagnosis than auscultation.
- 41. Treatment of Rheumaticfever :- A) Counselling, General measures & bed rest. B) Treatment of group A streptococcal infection. C) Anti-inflammatory therapy. D) Management of special condition- if present e.g. - Heart failure, Chorea etc.
- 42. A) General measures& bed rest: Table: Guide for bed rest & ambulation in pt with ARF Diet- Normal but salt & water restriction (if heart failure) Cardiac status Bed rest Gradual ambulation No carditis 2 wk 2 wks Carditis, no cardiomegaly 4 wks 4 wks Carditis with cardiomegaly 6 wks 6 wks Carditis, heart failure Strict bed rest for as long as heart failure is present & gradual ambulation for 3 month
- 43. It ispreferable to ensure adequate rest till the following clinical & laboratory criteria are met: 1) Normal body temperature. 2) Disappearance of joint symptoms and signs. 3) Absence of tachycardia at rest specially during sleep. 4) Disappearance of signs of heart failure. 5) Return of heart size to normal in chest x-ray 6) Return of ESR to less than 25 mm in 1st hour 7) C-Reactive protein becomes negative. 8) PR interval in ECG returns to normal or stabilized if it was prolonged. 9) Disappearance of subcutaneous nodule, if any.
- 44. B) Treatment ofstreptococcal infection- Inj.Benzathine penicillin G - If wt>30 kg 1.2 million U , wt ≤ 30 kg, 6 lac U given as single I/M dose. or Oral penicillin 250 mg 4 times daily for 10 days or If patient allergic to penicillin , Erythromycin - 40 mg/kg/day - 4 times daily - for 10 days.
- 45. C) Anti-inflammatory drugs: RecommendedAnti-inflammatory agents for ARF: Arthralgia: Analgesics only (paracetamol can be used) Arthritis only and/or carditis without cardiomegaly or CHF: Aspirin 50-70 mg/kg/day in 4divided doses PO for 3-5 days, followed by 50 mg/kg/day in 4 divided doses PO for 3 wk and half that dose for another 2-4 wk.
- 46. Anti-inflammatory drugs (contd……. Carditis and cardiomegally or CHF Should be treated with corticosteroids. Dose of prednisolone- 2 mg/kg/day in 4 divided doses for 2-3 wk followed by half the dose for 2-3 wk and then tapering of the dose by 5 mg/24 hr every 2- 3 days. When prednisone is being tapered, aspirin should be started at 50 mg/kg/day in 4 divided doses for 6 wk to prevent rebound of inflammation.
- 47. Treatment of specialcondition (if present) Treatment of Sydenham’s chorea: Phenobarbitone - 16-32 mg every 6-8 hourly PO. If phenobarbitone is ineffective , Haloperidol - 0.01-0.03 mg/kg/day 12 hourly PO. Chlorpromazine - 0.5mg/kg every 4-6 hourly PO. Erythema marginatum and subcutaneous nodules has no specific treatment.
- 48. Complications: • Arthritis andChorea of ARF resolve completely. • Long term sequele of ARF usually limited to the heart - Rheumatic valvular heart disease. Infective endocarditis.
- 49. Rheumatic valvular lesions Valvesinvolved % of cases Mitral valve alone 50 Mitral and aortic valves 40 Mitral, aortic and tricuspid 5 aortic valve alone 2 All other combinations 3
- 50. Prevention There are twocomponents for prevention of RHD. Improvement of socioeconomic 1. General measure Avoidance of overcrowding Health education. Primary prophylaxis 2. Drug prophylaxis Secondary prophylaxis
- 51. Prevention: • Primary Prophylaxis: refers to antibiotic treatment of the Strep. URTI to prevent an initial attack of RF (before the 9th day of symptoms of acute GAS Pharyngitis is highly effective.) • However, approximately 30% of patients with acute rheumatic fever do not recall a preceding episode of pharyngitis and did not seek therapy.
- 52. Prevention (contd…) Secondary Prophylaxis:refersto the prevention of colonization or infection of URT with GAS in individual who have already had a previous attack of ARF.
- 53. Secondary prevention (contd…) Duration of prophylaxis: • RF without carditis - 5 yr or until 21 yr of age , (whichever is longer) • RF with Carditis without residual heart disease - 10 yr or until 21 yr of age (whichever is longer) • RF with carditis with valvular heart disease - 10 yr or until 40yr of age (whichever is longer), sometimes life long prophylaxis
- 54. Prognosis: Prognosis of ARFdepend on the- 1. Clinical manifestation present at the time of initial episode. 2. Severity of the initial episode. 3. The presence of recurrences. • Approximately 70% of the pt with carditis during the initial episode of ARF recover with no residual heart disease. • The more severe the initial cardiac involvement, the greater the risk for residual heart disease.
- 55. Prognosis (contd…) • Pt.without carditis during the initial attack are unlikely to have carditis with recurrences. • Pt. with Carditis during the initial episode are likely to have carditis with recurrences. • The risk of permanent heart damage increases with each recurrences.