Acute Rheumatic Fever
Dr. Basem Enany, MD
Lecturer of Cardiology
 The initial illness is usually characterized by a sore
throat (pharyngitis) that may be followed, within
approximately 1...
Diagrammatic structure of the group A
beta hemolytic streptococcus
Capsule
Cell wall
Protein antigens
Group carbohydrate
P...
Evidence of AUTOIMMUNITY
INDUCED BY STREPT. ANTIGENS
 Gamma-globulins in sarcolemma
of myofibrils
 Circulating ab. to he...
 Not all of the serotypes of group A streptococci
can cause rheumatic fever. The rheumatogenic
serotypes are thought to i...
INCIDENCE
 20 to 50 per 100,000 /year during the period of 1940 to
1960 and declined to 1/100,000/year in 1970s.
 100/10...
CARDIOVASCULAR LESIONS
 MYOCARDIUM (ASCHOFF BODY)
 ENDOCARDIUM
 PERICARDIUM
EXTRACARDIAC LESIONS
 JOINTS
 SKIN
 LUNG...
•On pathological examination, the valves are thickened
and display rows of small vegetations along their apposing
surfaces...
Rheumatic
heart
disease.
Abnormal
mitral valve.
Thick, fused
chordae
Aortic valve showing active valvulitis. The valve is slightly thickened
and displays small vegetations – "verrucae"
•Stenotic mitral
valve seen from
left atrium.
•Both
commissures
are fused; the
cusps are
severely
thickened.
•The left atr...
Myocardial Aschoff body – the cells are large, elongated, with large
nuclei; some are multinucleate
Acute Rheumatic vegetations:
Chronic RHD:
 Valve leaflet
thickening.
 Shortening,
thickening and
fusion of
tendinous cords.
Fibrinous Pericarditis:
Jones Criteria (Revised) for Guidance in the
Diagnosis of Rheumatic Fever*
Major Manifestation Minor
Manifestations
Suppor...
Pitfalls in diagnosis
 John’s criteria is only a guideline
Problems with over diagnosis
 A minor illness is misdiagnosed...
ARTHRITIS
most common
 IN 70% OF CASES
 ACUTE MIGRATORY ASYMMETRIC
POLYARTHRITIS
 USUALLY LARGE JOINTS
 Involved joint...
PANCARDITIS
 IN 50% OF CASES
 MOST SERIOUS CAUSE OF MORBIDITY AND MORTALITY
 MAY BE THE ONLY MANIFESTATION OF ARF
 LEA...
Chest radiograph of an 8 year old patient with acute carditis
before treatment
Same patient after 4 weeks
Two-dimensional
color flow
Doppler image of
the left
ventricular inflow
of a patient with
mitral
regurgitation in
the four...
Two-dimensional parasternal long-axis view of a patient with mitral
stenosis, showing thickened valve cusps (arrow), with ...
 UNCOMMON (<10%), but most specific
 SMALL (0.5-2 cm.)
 PAINLESS FIRM DISCRETE AND FREELY
MOBILE
 ON EXTENSOR TENDONS ...
Subcutaneous nodule on the extensor surface of elbow of
a patient with acute RF
 Sydenham's chorea most frequently occurs
in children or adolescents between the ages
of 5 to 15.
 Affects females appro...
 LONG LATENT PERIOD: 1 to 6 months
 In most patientsacutely
 sudden, aimless, irregular, involuntary, jerky
movements ...
 RARE (5-10%)
 MACULAR NONPRURITIC RASH WITH A
SERPIGINOUS ERYTHEMATOUS BORDER
SURROUNDING NORMAL LOOKING SKIN
 BEGINS ...
Erythema marginatum on the trunk, showing erythematous lesions
with pale centers and rounded or serpiginous margins
LABORATORY STUDIES
ISOLATION OF STREPT.
(THROAT CULTURES)
 Throat culture render positive
results in approximately 25 % o...
STREPTOCOCCAL AB. TESTS
ANTIGEN
EXTRACELLULAR PRODUCT
• SREPTOLYSIN-O
• SREPTOKINASE
• HYALURONIDASE
• DEOXYRIBONUCLEASE -...
 positive ASOT occur only in 80 % of
streptococcal throat infection. However
sensitivity may be increased to 95 % if
AHT ...
OTHER INVESTIGATIONS
CXR
 CARDIC SIZE(CHF,EFFUSION)
 RHC. PNUEMONITIS
ECG
 SINUS TACHCARDIA
 PROLONGED P-R
 ARRHYTHMI...
DIFFERENTIAL DIAGNOSIS
POLYARTHRITIS
 JUVENILE RHEUMATOID: usually involves small joints of the
fingers and here the swel...
D.D. of CARDITIS
 Innocent murmurs: The common mistake is
misinterpreting the innocent basal ejection systolic murmur
or ...
Treatment
 Step I - primary prevention
(eradication of streptococci)
 Step II - anti inflammatory treatment
(aspirin,ste...
STEP I: Primary Prevention of Rheumatic Fever
(Treatment of Streptococcal Tonsillopharyngitis)
Agent Dose Mode Duration
Be...
Arthritis only Aspirin 75-100
mg/kg/day,give as 4
divided doses for 6
weeks
(Attain a blood level 20-
30 mg/dl)
Carditis P...
 Bed rest
 Treatment of congestive cardiac
failure: -digitalis,diuretics, ACEI
 Treatment of chorea:
-diazepam or halop...
STEP IV : Secondary Prevention of Rheumatic Fever
(Prevention of Recurrent Attacks)
Agent Dose Mode
Benzathine penicillin ...
Duration of Secondary Rheumatic Fever
Prophylaxis
Category Duration
Rheumatic fever with carditis and At least 10 y since ...
For those who receive salicylate
therapy, blood levels and liver
function must be regularly
monitored (i.e., with blood an...
SYDENHAM’S CHOREA
 PHYSICAL & MENTAL REST
 As Sydenham's chorea may spontaneously
resolve or not cause significant funct...
 First-line therapy with anticonvulsant
medication: valproate sodium
(Depakene®) may be beneficial
 Carbamazepine has al...
 Dopamine antagonists are usually reserved for
those patients who fail to respond to valproate or
who present with severe...
 ARF IS THE MOST COMMON CAUSE OF
ACQUIRED HEART DISEASE IN CHILDREN
AND YOUNG ADULTS.
 DIAGNOSIS OF ARF SHOULD DEPEND ON...
0102229098
Basem_enany@yahoo.com
management of acute rheumatic fever
management of acute rheumatic fever
management of acute rheumatic fever
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management of acute rheumatic fever

  1. 1. Acute Rheumatic Fever Dr. Basem Enany, MD Lecturer of Cardiology
  2. 2.  The initial illness is usually characterized by a sore throat (pharyngitis) that may be followed, within approximately 1 to 5 weeks, by the sudden (acute) onset of rheumatic fever.  "latent period."  ARF is an inflammatory disease following group A streptococcal infection (i.e., sequelae) multiple tissues and organs (joints, skin, subcutaneous tissues, heart, and brain).
  3. 3. Diagrammatic structure of the group A beta hemolytic streptococcus Capsule Cell wall Protein antigens Group carbohydrate Peptidoglycan Cyto.membrane Cytoplasm …………………………………………… ……... Antigen of outer protein cell wall of GABHS induces antibody response in victim which result in autoimmune damage to heart valves, sub cutaneous tissue,tendons, joints & basal ganglia of brain
  4. 4. Evidence of AUTOIMMUNITY INDUCED BY STREPT. ANTIGENS  Gamma-globulins in sarcolemma of myofibrils  Circulating ab. to heart tissue.  No strept. can be found in lesions.
  5. 5.  Not all of the serotypes of group A streptococci can cause rheumatic fever. The rheumatogenic serotypes are thought to include 1, 3, 5, 6, 14, 18, 19, and 24.  Pharyngitis- produced by GABHS can lead to- acute rheumatic fever , rheumatic heart disease & post strept. Glomerulonepritis  Skin infection- produced by GABHS leads to post streptococcal glomerulo nephritis only. Group A Beta Hemolytic Streptococcus
  6. 6. INCIDENCE  20 to 50 per 100,000 /year during the period of 1940 to 1960 and declined to 1/100,000/year in 1970s.  100/100,000/year of ARF/RHD among the younger age group of the socially disadvantaged population. THE ATTACK RATE (INCIDENCE OF ARF IN PTS WITH STREPT. PHARYNGITIS)  3% OF UNTREATED PATIENTS  5-50% IN PTS WITH PREVIOUS ATTACKS EPIDEMIOLOGY  SOCIO-ECONOMIC STATUS  OUT BREAKS OF STREPT PHARYNGITIS
  7. 7. CARDIOVASCULAR LESIONS  MYOCARDIUM (ASCHOFF BODY)  ENDOCARDIUM  PERICARDIUM EXTRACARDIAC LESIONS  JOINTS  SKIN  LUNGS AND PLEURA  CNS
  8. 8. •On pathological examination, the valves are thickened and display rows of small vegetations along their apposing surfaces •Inflammation of the valves consists of oedema and mononuclear cell infiltration of the valvular tissue and the chordae tendineae in the acute phase; fibrosis and calcification occur with maintenance of the inflammatory process. •Myocarditis is characterised by infiltration of mononuclear cells, vasculitis and degenerative changes of the interstitial connective tissue. •The pathognomonic lesion is the Aschoff body in the proliferative stage, present in 30 to 40 per cent of biopsies of patients with acute RF
  9. 9. Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae
  10. 10. Aortic valve showing active valvulitis. The valve is slightly thickened and displays small vegetations – "verrucae"
  11. 11. •Stenotic mitral valve seen from left atrium. •Both commissures are fused; the cusps are severely thickened. •The left atrium is huge.
  12. 12. Myocardial Aschoff body – the cells are large, elongated, with large nuclei; some are multinucleate
  13. 13. Acute Rheumatic vegetations:
  14. 14. Chronic RHD:  Valve leaflet thickening.  Shortening, thickening and fusion of tendinous cords.
  15. 15. Fibrinous Pericarditis:
  16. 16. Jones Criteria (Revised) for Guidance in the Diagnosis of Rheumatic Fever* Major Manifestation Minor Manifestations Supporting Evidence of Streptococal Infection Clinical LaboratoryCarditis Polyarthritis Chorea Erythema Marginatum Subcutaneous Nodules Previous rheumatic fever or rheumatic heart disease Arthralgia Fever Acute phase reactants: Erythrocyte sedimentation rate, C-reactive protein, leukocytosis Prolonged P- R interval Increased Titer of Anti- Streptococcal Antibodies ASO (anti-streptolysin O), others Positive Throat Culture for Group A Streptococcus Recent Scarlet Fever *The presence of two major criteria, or of one major and two minor criteria, indicates a high probability of acute rheumatic fever, if supported by evidence of Group A streptococcal nfection. Recommendations of the American Heart Association
  17. 17. Pitfalls in diagnosis  John’s criteria is only a guideline Problems with over diagnosis  A minor illness is misdiagnosed as ARF  unnecessarily therapy  cardiac neurotic Problems with under diagnosis  another disease treatment for a non existent disease  No long term prophylaxis
  18. 18. ARTHRITIS most common  IN 70% OF CASES  ACUTE MIGRATORY ASYMMETRIC POLYARTHRITIS  USUALLY LARGE JOINTS  Involved joint is swollen and exquisitely painful and tender.  RESOLVES WITHIN 1-3 WEEKS  RESPONDS QUICKLY TO SALICYLATES, this may be taken as a therapeutic test  LEAVES NO PERMENANT DAMAGE
  19. 19. PANCARDITIS  IN 50% OF CASES  MOST SERIOUS CAUSE OF MORBIDITY AND MORTALITY  MAY BE THE ONLY MANIFESTATION OF ARF  LEAVES PERMENANT DAMAGE Rheumatic carditis is pancarditis and endocardium is almost always involved. Hence without murmur carditis cannot be diagnosed. MYOCARDITIS: TACHYCARDIA,ARRHYTHMIAS,A-V BLOCKS, CARDIOMEGALY, CHF ENDOCARDITIS: MR,AR,TR,PR (STENOTIC LESIONS ONLY AFTER MONTHS OR YEARS) With severe cardiac failure and pericardial effusion murmur may not be audible but in such cases the patient is usually very ill. PERICARDITIS:DRY OR WITH EFFUSION.NEVER ALONE.
  20. 20. Chest radiograph of an 8 year old patient with acute carditis before treatment
  21. 21. Same patient after 4 weeks
  22. 22. Two-dimensional color flow Doppler image of the left ventricular inflow of a patient with mitral regurgitation in the four-chamber view (top panel) and two- dimensional parasternal long- axis view (lower panel), showing lack of apposition of the leaflets of the mitral valve during systole (arrow)
  23. 23. Two-dimensional parasternal long-axis view of a patient with mitral stenosis, showing thickened valve cusps (arrow), with poor leaflet separation in diastole. Left atrium is enlarged, with a thrombus in the posterior aspect of it. Aortic valve is also stenotic
  24. 24.  UNCOMMON (<10%), but most specific  SMALL (0.5-2 cm.)  PAINLESS FIRM DISCRETE AND FREELY MOBILE  ON EXTENSOR TENDONS OF JOINTS  OCCASIONALLY ON SCALP AND SPINE  The subcutaneous nodules tend to appear after the first weeks of the disease course and usually disappear within a week or two. Subcutaneous nodules
  25. 25. Subcutaneous nodule on the extensor surface of elbow of a patient with acute RF
  26. 26.  Sydenham's chorea most frequently occurs in children or adolescents between the ages of 5 to 15.  Affects females approximately twice as frequently as males, particularly in the years around puberty. As a result, some researchers suggest that sex hormones (e.g., the female hormone estrogen) may play some role in the development of the syndrome. CHOREA
  27. 27.  LONG LATENT PERIOD: 1 to 6 months  In most patientsacutely  sudden, aimless, irregular, involuntary, jerky movements   A significant deterioration in handwriting (in school-aged children)  Slight or significant difficulties dressing, feeding, and walking  Slurred, slowed speech (dysarthria)  disappear with sleep and may increase with stress, fatigue, excitement, or other factors.  Bilateral (20% hemichorea)  emotional or behavioral abnormalities  spontaneously resolve within approximately 3 to 6 months  However, in some instances, there may be residual signs of chorea and behavioral abnormalities, which may wax and wane over a year or more
  28. 28.  RARE (5-10%)  MACULAR NONPRURITIC RASH WITH A SERPIGINOUS ERYTHEMATOUS BORDER SURROUNDING NORMAL LOOKING SKIN  BEGINS AS RED OR PINK MACULES THAT FADE CENTRALLY  ON TRUNK & PROXIMAL EXTREMITIES  NEVER FACE AND HANDS  ABOUT 1INCH IN DIAMETER  This skin rash tends to appear early in the disease course, may persist or recur when other symptoms have subsided, and usually only affects patients with carditis.
  29. 29. Erythema marginatum on the trunk, showing erythematous lesions with pale centers and rounded or serpiginous margins
  30. 30. LABORATORY STUDIES ISOLATION OF STREPT. (THROAT CULTURES)  Throat culture render positive results in approximately 25 % of children of ARF probably related to early antibiotic administration.  -VE(75% OF PTS.)  FALSE +VE: Positive throat culture need not indicate infection because positive throat culture may occur in carrier state as in many school going children.
  31. 31. STREPTOCOCCAL AB. TESTS ANTIGEN EXTRACELLULAR PRODUCT • SREPTOLYSIN-O • SREPTOKINASE • HYALURONIDASE • DEOXYRIBONUCLEASE -N • NICOTINAMIDE ADENINE DINUCLEOTIDASE • ALL OF THE ABOVE CELLULAR COMPONENT • TYPE-SPECIFIC M PROTEIN • GROUP-SPECIFIC POLYSACCHARIDE TEST  ANTI-STREPTOLYSIN-0  ANTI-STREPTOKINASE  ANTI-HYALURONIDASE  ANTI-DNAse B  ANTI-NADase  STREPTOZYME  TYPE-SPECIFIC AB.  ANTI-A CARBOHYDRATE
  32. 32.  positive ASOT occur only in 80 % of streptococcal throat infection. However sensitivity may be increased to 95 % if AHT and anti DN ase B are also tested.
  33. 33. OTHER INVESTIGATIONS CXR  CARDIC SIZE(CHF,EFFUSION)  RHC. PNUEMONITIS ECG  SINUS TACHCARDIA  PROLONGED P-R  ARRHYTHMIAS  ST-T CHANGES ECHOCARDIOGRAPHY  MYOCARDIAL(DILATATION,FAILURE)  PERICARDIAL(EFFUSION)  ENDOCARDIAL(VALVULAR AFFECTION)
  34. 34. DIFFERENTIAL DIAGNOSIS POLYARTHRITIS  JUVENILE RHEUMATOID: usually involves small joints of the fingers and here the swelling is disproportionate to the symptom and usually the manifestation takes a longer time to subsides and residual deformity is common.  ‘Growing pains’ of children is mistaken for arthritis. But the symptom is not over the joints, pain is severe at night and the child is well during the day time.  SLE  MIXED COLLAGEN DSE.  POST-INFECTIOUS REACTIVE  INFECTIVE  SERUM SICKNESS
  35. 35. D.D. of CARDITIS  Innocent murmurs: The common mistake is misinterpreting the innocent basal ejection systolic murmur or left parasternal systolic murmur (Still’s) as evidence of carditis since they are misinterpreted for mitral regurgitation. Still’s murmur is vibratory in quality, usually late systolic unlike the systolic murmur of carditis which is usually pansystolic or occupies most of systole. The quality is also different from Still’s murmur. Isolated ejection systolic murmurs shall never be taken as evidence of carditis.  Tachycardia associated with fever and anxiety may be misinterpreted as evidence of myocarditis. This can be avoided if one pays attention to sleeping pulse rate.  INFECTIVE ENDOCARDITIS  COLLAGEN DSE.(SLE,KAWASAKI)  VIRAL MYOCARDITIS/pericarditis
  36. 36. Treatment  Step I - primary prevention (eradication of streptococci)  Step II - anti inflammatory treatment (aspirin,steroids)  Step III- supportive management & management of complications  Step IV- secondary prevention (prevention of recurrent attacks)
  37. 37. STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis) Agent Dose Mode Duration Benzathine penicillin G 600 000 U for patients< IM Once 27 kg (60 lb) 1 200 000 U for patients >27 kg or Penicillin V Children: 250 mg 2-3 times daily Oral 10 d (phenoxymethyl penicillin) Adolescents and adults: 500 mg 2-3 times daily For individuals allergic to penicillin Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d (maximum 1 g/d) Recommendations of American Heart Association
  38. 38. Arthritis only Aspirin 75-100 mg/kg/day,give as 4 divided doses for 6 weeks (Attain a blood level 20- 30 mg/dl) Carditis Prednisolone 2-2.5 mg/kg/day, give as two divided doses for 2 weeks Taper over 2 weeks & while tapering add Aspirin 75 mg/kg/day for 2 weeks. Continue aspirin alone 100 mg/kg/day for another 4 weeks Step II: Anti inflammatory treatment Clinical condition Drugs
  39. 39.  Bed rest  Treatment of congestive cardiac failure: -digitalis,diuretics, ACEI  Treatment of chorea: -diazepam or haloperidol  Rest to joints & supportive splinting 3.Step III: Supportive management & management of complications
  40. 40. STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) Agent Dose Mode Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular or Penicillin V 250 mg twice daily Oral or Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral 1.0 g once daily for patients >27 kg (60 lb) For individuals allergic to penicillin and sulfadiazine Erythromycin 250 mg twice daily Oral *In high-risk situations, administration every 3 weeks is justified and recommended Recommendations of American Heart Association
  41. 41. Duration of Secondary Rheumatic Fever Prophylaxis Category Duration Rheumatic fever with carditis and At least 10 y since last residual heart disease episode and at least until (persistent valvar disease*) age 40 y, sometimes lifelong prophylaxis Rheumatic fever with carditis 10 y or well into adulthood, but no residual heart disease whichever is longer (no valvar disease*) Rheumatic fever without carditis 5 y or until age 21 y, whichever is longer *Clinical or echocardiographic evidence. Recommendations of American Heart Association
  42. 42. For those who receive salicylate therapy, blood levels and liver function must be regularly monitored (i.e., with blood and urine tests) to help reduce the possibility of salicylate toxicity, a condition that may be characterized by headache, rapid breathing (tachypnea), vomiting, irritability, reduced levels of sugar in the blood (hypoglycemia), and/or other findings.
  43. 43. SYDENHAM’S CHOREA  PHYSICAL & MENTAL REST  As Sydenham's chorea may spontaneously resolve or not cause significant functional impairment, many experts indicate that treatment should be avoided unless associated chorea is functionally disabling or associated with potentially violent flailing motions of the limbs that may result in self-injury.
  44. 44.  First-line therapy with anticonvulsant medication: valproate sodium (Depakene®) may be beneficial  Carbamazepine has also been suggested as a first-line treatment for Sydenham’s chorea.
  45. 45.  Dopamine antagonists are usually reserved for those patients who fail to respond to valproate or who present with severe forms (i.e., chorea paralytica).  Haloperidol (initial dose of 0.5 to 1mg/kg/day, maximum, 5mg/day)  If fails, the next steps may include immunomodulatory treatment, steroids, IV IgG, or plasmapheresis.  Treatment is usually maintained for 8-12 weeks.
  46. 46.  ARF IS THE MOST COMMON CAUSE OF ACQUIRED HEART DISEASE IN CHILDREN AND YOUNG ADULTS.  DIAGNOSIS OF ARF SHOULD DEPEND ON CLINICAL,LABORATORY & IMAGING INVESTIGATIONS.  TREATMENT OF CARDITIS WITH SALICYLATES , STEROIDS.  LONG-TERM PROPHYLAXIS WITH LONG ACTING PCN. IS HIGHLY RECOMMENDED.
  47. 47. 0102229098 Basem_enany@yahoo.com
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