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CARDIOLOGY > Acute Rheumatic fever > Flashcards

Acute Rheumatic fever Flashcards

1
Q

ARF

A

Autoimmune inflammatory nonsupurative process that develops as a sequela of a streptococcal throat infection ( group A betahemolytic Strep) after 2-6 weeks , affecting different structures of conjuctive tissue :

  • HEART
  • SUBCUTANEOUS TISSUE
  • JOINTS
  • CNS
  • SKIN
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2
Q

Rheumatic heart disease

A

Chronic damage of cardiac valves ( typically MITRAL STENOSIS)
- can be prevented and controlled

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3
Q

ARF - age

A

5-15 years –> Children
Incidence has steadily in the last 50 years
developed countries - 1/100000
developing countries 200/100000

Reccurence rate is high in 1st year after diagnosis 50%

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4
Q

AFR = Most frequent cause of

A

Acquired cardiac failure in children

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5
Q

Risk factors

A
  • Age
  • Untreated Strep throat
  • Familial predisposition
  • Crowding
  • Poverty
  • Lack of medical care
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6
Q

Etiology

A

Group A beta hemolytic streptococci :
- 80 strains
Gram positive bacteria / encapsulated / resistance to phagocytosis
Classical rheumatogenic strains emm types : 1,3,5,6,18 -> expression of its surface antigen - M protein
New data : Any strain can lead to streptococcal pharyngitis + ARF -new reinfections with different serotypes

Direct and toxic infectious hypothesis - EXCLUDED

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7
Q

Pathogenesis

A
  • Abnormal ANTIGEN - ANTIBODY immune reaction

- Cross reaction b/w Streptococcal antigens and human tissular proteins - molecular mimicry

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8
Q

Immune Mechanism in ARF

A
  • Increased specific HUMORAL immune response: There is no streptococcal genetic material in rheumatic heart lesion

The rheumatogenic serotypes : M protein antigen and Nacetilglucosamine will lead to an increased humoral response - specific autoantibodies to these tissues

  • Myocardium ( against myosin + tropomyosin )
  • Endothelium and valvular endocardium ( antilaminine)
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9
Q

Immune Mechanism in ARF

Extracellular antigens of rheumatic streptococci

A
  • O and S hemolysins - streptolysins
  • Treptokinase enzymes
  • Hyaluronidase enzymes
  • DNA - ases (deoxyribonucleases)
    They are not pathogenic , are used only in diagnostic purposes

It is used frequently:
ASLO = Titration test of anti-streptolysin O antibodies

Cellular Immune response:
- Role in persistence of granulomatous lesions and aggravation of valvular lesions

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10
Q

Histological elements

A
  1. Exsudative phase
    - conjuctive tissue edema
    - collagen fibers edema
    - inflammatory infiltrate
    - fibrinoid necrosis

2.Granulomatous - proliferative phase (1-6 mnths)
ASCHOFF NODULES = pathognomonical
= Central necrosis + Multicellular surrounding with plurinuclear cells

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11
Q

Anitskchow cells

A

Multinucleated giant cells = macrophages from Aschoff nodules with the appearance of ‘‘owl’s eyes’’ or like a ‘‘catterpillar’’ ( chromatine)

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12
Q

SOS SOS SOS

Clinical presentation RF

A

MAJOR MANIFESTATIONS:

  • Sydenham Chorea
  • Polyarthritis
  • Erythrema marginatum
  • Subcutaneous nodules
  • Carditis
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13
Q

Arthritis

75 - 80 %

A

typically polyarticular , but monoarthritis may occur with ARF in select high-risk populations

  • 6-16 joints
  • large joints - knees ,ankles,elbows,wrists
  • very painful
  • usually symmetrical
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14
Q

Arthritis

Migratory pattern

A
  • 3-7 days until resolution and then appears the inflammation of another joint
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15
Q

Arthritis

TREATMENT

A

HIGH DOSE OF SALICYLATES
- Diagnostic test : clear improving or resolution of inflammation at 24hrs after aspirin initiation
The entire bout of arthritis subsides within 4-6 weeks without any permanent damage. If not , a different diagnosis should be considered

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16
Q

ARTHRALGIA -

A

MINOR CRITERIA

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17
Q

Arthritis associations

A

FREQUENTLY ASSOCIATES WITH CARDITIS !!!

Rheumatic fever licks at the joints , but bites at the heart

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18
Q

Subcutaneous nodules

20%

A 
Late manifestation-
after 6 wks of RF evolution 
Painless subcutaneous nodules :
- 0.5 -2 cm
- firm
-symmetric
On the extension areas and on the bony protruberences
Complete resolution
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19
Q

Erythema marginatum

5%

A
  • Early sign
  • nonpruritic , painless, serpiginous, erythrematous eruption on the trunk ( macular , with pale central area )
  • centrifugal extension
  • trunk and proximal limbs
  • they get worse with heat application
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20
Q

SYDENHAM CHOREA

20%

A
  1. Late neurological manifestation - over 3 months
  2. Autoantibodies reacting with brain ganglioside
    - caudate nuclei
    - thalamus
  3. Involuntary limb movements , incoordination, speech disorders , facial grimaces
  4. More common in girls
  5. Self limited ,2-6 wks, complete resolution
  6. INCREASED RISK OF RF RECURRENCE - PHROPHYLAXIS MANDATORY
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21
Q

CARDITIS

50%

A

PANCARDITIS
Early onset aafter arthritis
Clinical signs :
- High pulse rate
Murmur - mitral /aortic regurgitation - endocardium involved
- Cardiomegaly - myocardium involvement
-Pericardial friction rub - Pericarditis
-Prolonged PR interval - myocardial inflammation affecting electrical conduction
- Cardiac failure

22
Q

Endocarditis

A

Active inflammation of the heart tissues : valvular lesions
- exudative - proliferative , fibrinoid necrosis , +- vegetations
Acute onset -> Valve regurgitation
New murmurs / modifications of pre-existing ones
1.MITRAL SYSTOLIC MURMURS - 70 -80% of patients
- functional + organic
( edema,vegetations,papillary muscles ,hypotonia)

  1. CAREY-COOMBS MURMUR - Apical dyastolic murmur ( rapid filling of LV - increased blood flow across a thickened mitral valve )
  2. AORTIC DIASTOLIC MURMUR -10%

They disappear in evolution - STENOSES APPEAR LATE

23
Q

Endocarditis

Chronic stages

A
  • scars +-calcific lesions

Progressive destruction of the valvular apparatus

STENOSIS:

  • Annulus tightening
  • Commisurral fusions
  • Chordal shortening/thickening
  • Leaflet thickening - restricted motion
24
Q

VALVE INVOLVEMENT IN RHD

A

STENOSIS :
Valve doesnt openall the way , not enough blood passes through

REGURGITATION:
Valve doesnt close all the way so blood leaks backwards

25
Q

MYOCARDITIS

A

Myocardial lesions:

  • Focal myocarditis with rheumatic nodules : oligosymptomatic
  • Interstitial diffuse myocarditis : Acute HF
  • Infiltration of conduction tissue : AV conduction disorders
26
Q

MYOCARDITIS

CLINICAL

A
  • Symptoms of acute left ventricular failure dyspnea
  • > Acute pulm edema
  • Tachycardia
  • Rhythm disorders ( PB->sustained VT)
  • Gallop S3 ( severe myocardial damage)
  • functional murmurs - annular dilatation
  • Cardiomegaly ( clinical /radiological )
27
Q

PERCARDITIS

A

10%
NOT CONSTRICTIVE
Fibrinous pericarditis
Bread and butter pericarditis

28
Q

PERICARDITIS

A 
1.FIBRINOUS PERICARDITIS 
Significant anterior chest pain , accentuated by inspiration , changes position 
- Pericardial rub 
2.EXUDATIVE PERICARDITIS 
Less severe pain / chest pressure 
-Anxiety 
- Mohammedan prayer sign 
Cardiac tamponate (rare)
29
Q

Positive DIAGNOSIS OF RHEUMATISMAL CARDITIS

A
  1. New cardiac murmur ( Echo)
  2. Cardiac dilation
  3. Congestive cardiac failure
  4. Pericardial rub
30
Q

MINOR CLINICAL CRITERIA

A

FEVER
acute phase
very responsive to NSAIDs

ARTHRALGIA

31
Q

PARACLINICAL

LABORATORY

A
  • Acute inflammatory syndrome + direct and indirect evidence of
  • recent streptococcal infection
32
Q

BLOOD ANALYSES

A

ESR and CRP
- minor criteria
- nonspecific
low in those with isolated chorea /NSAIDs
Inactive disease when ESR and PCR return to normal ( and remains N over 2 weeks)
These analyses usually remains increased even after complete clinical resolution

CRP is more specific ; it remains approximately 3 months , increased or even more in those with valvular damage ( 5% over 6 months )

33
Q

EVIDENCE OF STREPTOCOCCAL INFECTION

A 
1.Positive pharyngeal throat culture during streptococcal angina 
Pbs : carries ( absent ...
2.Elevated or ...
ASLO > 500 - 600 U 
Maximum values 
Anti-DNA B ( 20% ...)

They remain raised for a long time ( chorea ) ; used when ASLO inconclusive

3.Rapid streptococcal sarbohydrate antigen test
90-100% specificity , less sensitivity ( 70%)
Use for prevention of ARF

34
Q

MAJOR JONES CRITERIA

A 
J Joints ( polyarthritis)
O Carditis (pancarditis)
N nodules 
E erythema marginatum
S Sydenham's chorea
35
Q

MINOR JONES CRITERIA

A

Clinical

Acute phase reactants ( ESR,CRP)
PR interval prolonging ( ECG)

36
Q

DIAGNOSIS

A 
2 MAJOR CRITERIA
1 MAJOR + 2 MINORS 
!!! AND EVIDENCE OF STREPTOCOCCAL INFECTION 
specificity - 97%
sensitivity - 77%
37
Q

2003 WHO CRITERIA ( based on JONES criteria )

A
  • A primary episode of RF
  • Recurrent attacks of RF in patients without RHD
  • Recurrent attacks of RF in patients with RHD
  • Rheumatic chorea
  • Insidious onset rheumatic carditis
  • Chronic RHD
38
Q

Differential diagnosis

A
  1. Infectious ( bacterial ) endocarditis
  2. Septic polyarthritis
  3. Gonococcal arthritis
  4. SLE
  5. Rheumatoid arthritis
  6. Tuberculous arthritis
  7. Arthrus’s reaction to Penicillin
39
Q

Evolution and Prognosis

A

RELAPSE
recurrence of clinical / paraclinical signs of the disease upon discontinuation of treatment ( rebound)

RECURRENCE
new episode of ARF after a new streptococcal infection (with another serotype)
more than 2 months after stopping treatment

Maximum frequency in the first year , increased incidence in the first 5 years

40
Q

RECURRENCE

A
  1. Younger age at onset of ARF - increased risk of recurrence
  2. It decreases over time
  3. Recurrence repeats the clinical pattern of the first ARF attack
  4. More common in those with valvular lesions -> Mitral stenosis as first diagnosis of RHD suggests evolution with recurrences
  5. Highly increased antibody titer = high chances of recurrence
41
Q

PROGNOSIS

A
  • Vey good for those with extracardiac disorders
  • Relatively good for those without valvular sequelae
  • Reserved for those with valvular sequelae
    50% of those with carditis without prophylaxis
    10-20% years after the first ARF attack
42
Q

Chronic rheumatic disease

A
  • the result of repeated valvulitis after repeated episodes of ARF
  • Multivalvular , left heart generally affected
  • Mitral - aortic - tricuspid - pulmonar

65-70% - 20-25% -10% - rarely

43
Q

Valvular chronic rheumatic disease

A
  • Valve thickening
  • Commissural fusion
  • Shortening restricting , thickening of chordae
    !!! Mitral stenosis as a chronic condition in adults ( regurgitation in children)
44
Q

Mitral stenosis

the differences

A

The normal valve :

  • Transparent
  • Avascular
  • Thin
  • Flexible membrane

RHD:

  • Thick
  • Fibrous scarred stenotic & fixed (MS/MR) with blood vessels
45
Q

TREATMENT

A 
ARF treatment 
- symptomatic of acute manifestations 
-eradication of streptococcal infection 
Recurrence prevention 
- primary prophylaxis
- secondary prophylaxis
46
Q

CURATIVE TREATMENT

A

A.Eradication of streptococcal infection :
- Penicillin V250 mg ( children ) or 500 mg (adults), splitted in 2-3 doses/day,po,10 days

  • Benzatin penicilin (Moldamin ) 600000 unit. im for children and 1.2 mil unit. for adults single injection-compliance
  • For patients allergic to penicillines : Erythromycin oral 1.6-2 g/24hrs , 10 days
  • Or oral Cephalosporins
47
Q

CURATIVE TREATMENT

A.Eradication of streptococcal infection :

A
  • Penicillin V250 mg ( children ) or 500 mg (adults), splitted in 2-3 doses/day,po,10 days
  • Benzatin penicilin (Moldamin ) 600000 unit. im for children and 1.2 mil unit. for adults single injection-compliance
  • For patients allergic to penicillines : Erythromycin oral 1.6-2 g/24hrs , 10 days
  • Or oral Cephalosporins
48
Q

CURATIVE TREATMENT

B.Anti- inflammatory treatment

A

ARF without carditis :

  • Salicylates - Aspirin :6-8 g /24hrs ( 100-125 mg/kg/day for children over 12 yo) , gradually decreased in 6-8 wks
  • Steroids are also effective but should probably be reserved for patients in whom salicylates fail

ARF with carditis :
From the beginning Prednisone 1-2 mg/kg/day 2-4 wks,overlap with Aspirin low doses in the last 2 wks ;
Prednisone has to be dcreased by 5mg every 4-5 days and continued only Aspirin to prevent relapse

ARF with chorea : sedatives , haloperidol , isolation

49
Q

PROPHYLACTIC TREATMENT

Primary prevention

A
  • Prompt recognition of pharyngotonsillitis plus treatment ( in the fist 10 days)

Benzathin benzylpenicillin (Moldamin) 1.2 mil u im single dose or

Penicilline V oral , 10 days
For penicilline allergic pts:
- Erythromycin 40 mg/kg/day x 2-4 hrs per day,oral ,10 days
- Cephalosporins I and II ( Cefuroxime) , 10 days

50
Q

PROPHYLACTIC TREATMENT
SECONDARY PREVENTION
!!! As early as possible after ARF

A
  • Benzhatin penicilline ( Moldamin) 1.2 mil u /lmonth , im
  • ARF with a history of carditis + rheumatic valvulopathy
  • minimum 10 years , until 40 yo or indefinite/for life
  • ARF with carditis without valvulopathy
  • minimum 10 years or until 21 yo
  • RAA without carditis : minimum 5 years or until 21 yo

NOT TO GIVE TETRACYCLINE : RESISTANCE OVER 50%
Research for a Vaccine against Str.group A

CARDIOLOGY (19 decks)

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