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d
                n Zia
      Dr Irfa
By:




FEVER
&KNEE PAIN
AN 11-YEAR OLD
PRESENTED WITH
FEVER
JOINT PAIN
AND SWELLING
SOB
O/E : tachypnea and tachycardia
             T 38.2
             P 160
             RR 32
           BP 100/60
            SaO2 94%
Lungs clear
CVS S1S2, pansystolic murmur 3/6
 heard at apex with radiation to
              axilla
Abdomen Soft, normoactive bowel
             sounds
Left knee is swollen and extremely tender with
warmth & Limited ROM. His right ankle is very swollen
and warm. He has limited subtalar motion. Both his
knee and ankle are very tender even to touch. 
Enlarged, erythematosus tonsils with exudates
CNS : No abnormal
   movements of arms,
   hands, or tongue are
noted. He is unable to walk
        due to pain.
P   R
CARDIOMEGALY
Diffe
      r   entia
                l   s?
RHEUMATIC
FEVER

       DR IRFAN ZIAD
12 million
are affected by ARF and
CRHD globally

developing countries are

150 times
more susceptible than
the developed countries


5-14 years
is the most frequent age
group
AN INFLAMMATORY DISEASE OF
CHILDHOOD RESULTING FROM
        UNTREATED
    STREPTOCOCCOCCAL
  PHARYNGEAL INFECTION
Group A
β-hemolytic
Streptococcus
Rheumatogenic strain
Group A B-hemolytic
                  Streptococcus
                  Rheumatogenic strain

                   tein
                Pro
           M-
                     Molecular
                     mimicry

Immune            Heart Brain Skin
response          Connective tissue
Joints
-Athralgia
-Migratory polyarthritis
Skin
-Subcutaneous nodules
-Erythema marginatum
Subcutaneous nodules
Erythema marginatum
CNS
Basal Ganglia
syndenham
CHOREA
aka San Vitus Dance
rapid involuntary
movement affecting
the hands, feet,
tongue and face.
Heart
Myocardium
Pericardium
Endocardium
Pericardium
Pancarditis   Myocardium
              Endocardium
Myocarditis
-heart is enlarged
-may develop CCF
Pericarditis
- Completely resolved
- Heal with adhesion
  Myocardium

 Endocardium


“Bread and Butter”
 Pericarditis
Myocardium
Endocardium
In patients with critical stenosis, mitral
valvulotomy, percutaneous balloon valvuloplasty,
  or mitral valve replacement may be indicated.
polyarthritis
erythema marginatum & nodules
Sydenham chorea
Rheumatic myocarditis & pericarditis
Rheumatic endocarditis
MODIFIED JONES CRITERIA FOR
    ACUTE RHEUMATIC FEVER
    MAJOR CRITERIA        MINOR CRITERIA
Carditis                Fever (Temp>38oC)
Polyarthritis, aseptic  ESR > 30 mm/h or
monoarthritis or        CRP > 30 mg/L
polyathralgia
Chorea                 Prolonged PR interval
Erythema marginatum
Subcutaneous nodules
INVESTIGATIONS

FBC: anaemia, leucocytosis
Throat swab
ASOT
CXR, ECG
Echocardiogram
How to make the diagnosis
      Initial episode of ARF

          2   MAJOR CRITERIA

                     OR
1   MAJOR CRITERIA
                     +2   MINOR CRITERIA

+ evidence of a preceding group
   A streptococcal infection
How to make the diagnosis
     Recurrent attack of ARF
          2   MAJOR CRITERIA

                     OR
1   MAJOR CRITERIA
                     +2   MINOR CRITERIA
                     OR
           3 of a preceding group A
              MINOR CRITERIA
     + evidence
          streptococcal infection
Evidence of a preceding group A
     streptococcal infection

Increased antistreptolysin O titre (ASOT)
Positive throat culture for GABHS
Positive rapid antigen detection test
J oints - artritis
       O bvious - cardiac
       N odules - rheumatic
 AJ OR
M      E rythema marginatum
       S ydenham chorea

      I nflammatory cells (leukocytosis)
      T emperature
    R E SR/CRP elevated
MINO R aised PR interval
      I tself (Previous Hx of Rheumatic fever)
      A thralgia
TREATMENT


  Bedrest
TREATMENT
   Anti-Streptococcal Therapy
   IV C. Penicillin 50 000U/kg/dose 6H
                      or
   Oral Penicillin V 250 mg 6H (<30kg),
      500 mg 6H (>30kg) for 10 days
Oral Erythromycin for 10 days if allergic to penicillin.
TREATMENT
Anti-Inflammatory Therapy
       mild / no carditis:
Oral Aspirin 80-100 mg/kg/day in 4 doses for
          2-4 weeks, tapering over
                  4 weeks.
TREATMENT
Anti-Inflammatory Therapy
Pericarditis, or moderate to severe
              carditis:
Oral Prednisolone 2 mg/kg/day in 2 divided
           doses for 2 - 4 weeks,
  taper with addition of aspirin as above.
TREATMENT
Anti-Inflammatory Therapy
    Monitor Aspirin level and LFT
Watch out for
REYES
SYNDROME
Encephalopathy
Fatty liver degeneration
Transaminase elevation.
TREATMENT
  Anti-failure medication
Diuretics, ACE inhibitors, digoxin (to be used
                with caution).
TREATMENT
      Secondary prophylaxis
• IM Benzathine Penicillin 0.6 mega units (<30 kg)
   or 1.2 mega units (>30 kg) every 3 to 4 weeks.

       • Oral Penicillin V 250 mg twice daily.

• Oral Erythromycin 250 mg twice daily if allergic to
                     Penicillin.
TREATMENT
    Secondary prophylaxis
      Duration of prophylaxis
• Until age 21 years or 5 years after last attack of
            ARF whichever was longer
• Lifelong for patients with carditis and valvular
                   involvement.
Summary

        Accurate diagnosis is important.
       Jones Criteria is a good guideline
Management include post-strep therapy, anti-
inflammatory therapy, anti-failure therapy and
             secondary prophylaxis
ARF is a large physical, emotional and financial
                    burden.
In a case of knee swelling..




Don’t forget your stethoscope!
References
  1.  Kumar, Vinay; Abbas, Abul K; Fausto, Nelson; Mitchell, Richard N
  (2007), Robbins Basic Pathology (8th ed.), Saunders Elsevier,
  pp. 403–6, ISBN 978-1-4160-2973-1.
  2. "rheumatic fever" at Dorland's Medical Dictionary
  3. Jones, T Duckett (1944). "The diagnosis of rheumatic
  fever". JAMA 126(8):481. doi:10.1001/jama.1944.02850430015005.
  4. Ferrieri, P (2002). "Proceedings of the Jones Criteria
  workshop".Circulation (Jones Criteria Working Group) 106 (19):
  2521–3.doi:10.1161/01.CIR.0000037745.65929.FA. PMID 12417554.
  5. Parrillo, Steven J. "Rheumatic Fever". eMedicine. DO, FACOEP,
  FACEP. Retrieved 2007-07-14.
  6. Malaysian Paediatric Protocol, 3rd Edition

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Rheumatic fever

  • 1. d n Zia Dr Irfa By: FEVER &KNEE PAIN
  • 2. AN 11-YEAR OLD PRESENTED WITH FEVER JOINT PAIN AND SWELLING SOB
  • 3. O/E : tachypnea and tachycardia T 38.2 P 160 RR 32 BP 100/60 SaO2 94%
  • 4. Lungs clear CVS S1S2, pansystolic murmur 3/6 heard at apex with radiation to axilla Abdomen Soft, normoactive bowel sounds
  • 5. Left knee is swollen and extremely tender with warmth & Limited ROM. His right ankle is very swollen and warm. He has limited subtalar motion. Both his knee and ankle are very tender even to touch. 
  • 7. CNS : No abnormal movements of arms, hands, or tongue are noted. He is unable to walk due to pain.
  • 8. P R
  • 10. Diffe r entia l s?
  • 11. RHEUMATIC FEVER DR IRFAN ZIAD
  • 12. 12 million are affected by ARF and CRHD globally developing countries are 150 times more susceptible than the developed countries 5-14 years is the most frequent age group
  • 13. AN INFLAMMATORY DISEASE OF CHILDHOOD RESULTING FROM UNTREATED STREPTOCOCCOCCAL PHARYNGEAL INFECTION
  • 15. Group A B-hemolytic Streptococcus Rheumatogenic strain tein Pro M- Molecular mimicry Immune Heart Brain Skin response Connective tissue
  • 21. syndenham CHOREA aka San Vitus Dance rapid involuntary movement affecting the hands, feet, tongue and face.
  • 22.
  • 24. Pericardium Pancarditis Myocardium Endocardium
  • 26. Pericarditis - Completely resolved - Heal with adhesion Myocardium Endocardium “Bread and Butter” Pericarditis
  • 28.
  • 29. In patients with critical stenosis, mitral valvulotomy, percutaneous balloon valvuloplasty, or mitral valve replacement may be indicated.
  • 30. polyarthritis erythema marginatum & nodules Sydenham chorea Rheumatic myocarditis & pericarditis Rheumatic endocarditis
  • 31. MODIFIED JONES CRITERIA FOR ACUTE RHEUMATIC FEVER MAJOR CRITERIA MINOR CRITERIA Carditis Fever (Temp>38oC) Polyarthritis, aseptic ESR > 30 mm/h or monoarthritis or CRP > 30 mg/L polyathralgia Chorea Prolonged PR interval Erythema marginatum Subcutaneous nodules
  • 32. INVESTIGATIONS FBC: anaemia, leucocytosis Throat swab ASOT CXR, ECG Echocardiogram
  • 33. How to make the diagnosis Initial episode of ARF 2 MAJOR CRITERIA OR 1 MAJOR CRITERIA +2 MINOR CRITERIA + evidence of a preceding group A streptococcal infection
  • 34. How to make the diagnosis Recurrent attack of ARF 2 MAJOR CRITERIA OR 1 MAJOR CRITERIA +2 MINOR CRITERIA OR 3 of a preceding group A MINOR CRITERIA + evidence streptococcal infection
  • 35. Evidence of a preceding group A streptococcal infection Increased antistreptolysin O titre (ASOT) Positive throat culture for GABHS Positive rapid antigen detection test
  • 36. J oints - artritis O bvious - cardiac N odules - rheumatic AJ OR M E rythema marginatum S ydenham chorea I nflammatory cells (leukocytosis) T emperature R E SR/CRP elevated MINO R aised PR interval I tself (Previous Hx of Rheumatic fever) A thralgia
  • 38. TREATMENT Anti-Streptococcal Therapy IV C. Penicillin 50 000U/kg/dose 6H or Oral Penicillin V 250 mg 6H (<30kg), 500 mg 6H (>30kg) for 10 days Oral Erythromycin for 10 days if allergic to penicillin.
  • 39. TREATMENT Anti-Inflammatory Therapy mild / no carditis: Oral Aspirin 80-100 mg/kg/day in 4 doses for 2-4 weeks, tapering over 4 weeks.
  • 40. TREATMENT Anti-Inflammatory Therapy Pericarditis, or moderate to severe carditis: Oral Prednisolone 2 mg/kg/day in 2 divided doses for 2 - 4 weeks, taper with addition of aspirin as above.
  • 41. TREATMENT Anti-Inflammatory Therapy Monitor Aspirin level and LFT
  • 42. Watch out for REYES SYNDROME Encephalopathy Fatty liver degeneration Transaminase elevation.
  • 43. TREATMENT Anti-failure medication Diuretics, ACE inhibitors, digoxin (to be used with caution).
  • 44. TREATMENT Secondary prophylaxis • IM Benzathine Penicillin 0.6 mega units (<30 kg) or 1.2 mega units (>30 kg) every 3 to 4 weeks. • Oral Penicillin V 250 mg twice daily. • Oral Erythromycin 250 mg twice daily if allergic to Penicillin.
  • 45. TREATMENT Secondary prophylaxis Duration of prophylaxis • Until age 21 years or 5 years after last attack of ARF whichever was longer • Lifelong for patients with carditis and valvular involvement.
  • 46. Summary Accurate diagnosis is important. Jones Criteria is a good guideline Management include post-strep therapy, anti- inflammatory therapy, anti-failure therapy and secondary prophylaxis ARF is a large physical, emotional and financial burden.
  • 47. In a case of knee swelling.. Don’t forget your stethoscope!
  • 48. References 1.  Kumar, Vinay; Abbas, Abul K; Fausto, Nelson; Mitchell, Richard N (2007), Robbins Basic Pathology (8th ed.), Saunders Elsevier, pp. 403–6, ISBN 978-1-4160-2973-1. 2. "rheumatic fever" at Dorland's Medical Dictionary 3. Jones, T Duckett (1944). "The diagnosis of rheumatic fever". JAMA 126(8):481. doi:10.1001/jama.1944.02850430015005. 4. Ferrieri, P (2002). "Proceedings of the Jones Criteria workshop".Circulation (Jones Criteria Working Group) 106 (19): 2521–3.doi:10.1161/01.CIR.0000037745.65929.FA. PMID 12417554. 5. Parrillo, Steven J. "Rheumatic Fever". eMedicine. DO, FACOEP, FACEP. Retrieved 2007-07-14. 6. Malaysian Paediatric Protocol, 3rd Edition