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CARDIOLOGY > Acute Rheumatic fever > Flashcards

Acute Rheumatic fever Flashcards

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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%

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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

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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

Study These Flashcards
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

Study These Flashcards
A

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

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

25
MYOCARDITIS
Myocardial lesions: - Focal myocarditis with rheumatic nodules : oligosymptomatic - Interstitial diffuse myocarditis : Acute HF - Infiltration of conduction tissue : AV conduction disorders
26
MYOCARDITIS | CLINICAL
- 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
PERCARDITIS
10% NOT CONSTRICTIVE Fibrinous pericarditis Bread and butter pericarditis
28
PERICARDITIS
``` 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
Positive DIAGNOSIS OF RHEUMATISMAL CARDITIS
1. New cardiac murmur ( Echo) 2. Cardiac dilation 3. Congestive cardiac failure 4. Pericardial rub
30
MINOR CLINICAL CRITERIA
FEVER acute phase very responsive to NSAIDs ARTHRALGIA
31
PARACLINICAL | LABORATORY
- Acute inflammatory syndrome + direct and indirect evidence of - recent streptococcal infection
32
BLOOD ANALYSES
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
EVIDENCE OF STREPTOCOCCAL INFECTION
``` 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
MAJOR JONES CRITERIA
``` J Joints ( polyarthritis) O Carditis (pancarditis) N nodules E erythema marginatum S Sydenham's chorea ```
35
MINOR JONES CRITERIA
Clinical Acute phase reactants ( ESR,CRP) PR interval prolonging ( ECG)
36
DIAGNOSIS
``` 2 MAJOR CRITERIA 1 MAJOR + 2 MINORS !!! AND EVIDENCE OF STREPTOCOCCAL INFECTION specificity - 97% sensitivity - 77% ```
37
2003 WHO CRITERIA ( based on JONES criteria )
- 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
Differential diagnosis
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
Evolution and Prognosis
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
RECURRENCE
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
PROGNOSIS
- 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
Chronic rheumatic disease
- 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
Valvular chronic rheumatic disease
- Valve thickening - Commissural fusion - Shortening restricting , thickening of chordae !!! Mitral stenosis as a chronic condition in adults ( regurgitation in children)
44
Mitral stenosis | the differences
The normal valve : - Transparent - Avascular - Thin - Flexible membrane RHD: - Thick - Fibrous scarred stenotic & fixed (MS/MR) with blood vessels
45
TREATMENT
``` ARF treatment - symptomatic of acute manifestations -eradication of streptococcal infection Recurrence prevention - primary prophylaxis - secondary prophylaxis ```
46
CURATIVE TREATMENT
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
CURATIVE TREATMENT | 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
48
CURATIVE TREATMENT | B.Anti- inflammatory treatment
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
PROPHYLACTIC TREATMENT | Primary prevention
- 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
PROPHYLACTIC TREATMENT SECONDARY PREVENTION !!! As early as possible after ARF
- 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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