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REUMATISMUL

ARTICULAR ACUT

Definiie
Afeciune sistemica

inflamatorie
acut
nesupurativ
uneori recurenta

a esutului conjunctiv

Definiie
Caracteristici:
Clinic: consecin tardiv (perioad de laten de 3 sptmni) a

infeciei faringiene cu streptococ beta hemolitic de grup A

Anatomic: alterarea fibrilelor de colagen i a substanei tesutului

conjunctiv

Leziunile inflamatorii afecteaz mai multe organe: articulaii, inim,

tegumente, esut celular subcutanat i sistem nervos central

Importan: determin fibroz la nivelul aparatelor valvulare

cardiace, determinnd modificarea cronic a hemodinamicii cardiace

Frequency
Leading cause of death and common cause of structural heart
damage until 1960
US has experienced a resurgence of RF in the last two decades.
Why? Possibly more virulent strains of Group A streptococci.
ARF problem in high-risk areas of the tropics, countries with
limited resources and where there is minority indigenous
populations
Most cases = 5-15 years old
Rare <3 yrs
Girls>boys
Incidence more during fall ,winter & early spring
3% of untreated sorethroats Rheumatic fever
1/3rd result from inapperant infection

Changing incidence of ARF in Alaska in rural populations

Epidemiologie
superpozabil cu a faringitei streptococice:
1. factorul economic sau de mediu
2. factorul bacteriologic
3. factorul gazd

Epidemiologie

1. factorul economic sau de mediu:


condiiile de via necorespunztoare
malnutriia
supraaglomeraia

- frecven n rile dezvoltate

Epidemiologie
2. factorul bacteriologic:
streptococ beta hemolitic de grup A
(M-serotipurile, tipul 1, 3, 5, 6, 14, 18, 19, 27, i 29).
Tulpinile streptococice reumatogene:
coninut proteina M de suprafa
capsula bogat n acid hialuronic
formeaz colonii "mucoide" i manifest virulen exacerbat;
rezist la fagocitoz

-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

Epidemiologie

3. factorul gazd:
vrstele 5 - 15 ani
predispoziia genetic
recurena reumatismal
inciden egal brbai / femei

Etiopatogenie
infecia cu streptococ beta hemolitic de grup A
la nivelul cilor respiratorii superioare, n
antecedente

prezena infeciei

intensitatea rspunsului de
aprare al organismului

Etiopatogenie
Argumente pentru etiologia streptococic a RAA:
studii clinice i epidemiologice: faringita streptococic
perioad de laten de 3-6 sptmni RAA
studii imunologice: titrul crescut de anticorpi mpotriva unor
antigene streptococice
RAA i recurenele reumatismale pot fi prevenite prin tratamentul
antibiotic contra infeciei streptococice faringiene

Etiopatogenie

Mecanismul patogenic: reacie autoimun


mediat de anticorpi - indus de infecia
streptococic, prin intermediul anticorpilor cu
reactivitate incrucisata (cross-reaction).

Etiopatogenie
Argumente pentru reacia autoimun:
perioad de laten relativ lung debut faringit - apariia RAA
similitudine antigenic = constituenii somatici ai streptococului
de grup A - esuturile umane reacii imunologice ncruciate:
carbohidratul specific de grup coninut n peretele
celular streptococic ~ o glicoprotein de la nivelul valvelor
cardiace valvulita reumatismal
membrana celular streptococic ~ sarcolema
miocardic miocardita reumatismal
anticorpii cross-reactani mpotriva nucleului caudat
chorea Sydenham

Etiopatogenie

? caracterele genetice ale gazdei

Pathophysiology
In acute rheumatic fever, antibodies are mounted against N-acetyl-beta-Dglucosamine (NABG or GlcNAc), the immunodominant carbohydrate
antigen of group A streptococci.
These antibodies likely play a role in valvular injury in rheumatic carditis,
and in other manifestations of ARF.
In Sydenham chorea, the antibodies bind to lysoganglioside on the neuronal
cell surface, where they are capable of triggering a signaling cascade, and
also recognize the intracellular protein tubulin.
These tubulin-specific antibodies are not found in patients with acute
rheumatic fever but no SC, or in patients who have recovered from SC.
Moreover, the genes encoding these antibodies are similar to the genes
encoding antibodies implicated in the pathogenesis of motor neuropathies.
From a pathologic viewpoint, the available data are consistent with
involvement of the basal ganglia and cortical structures. In individual case
reports, magnetic resonance imaging studies performed during and after an
acute episode of SC showed reversible abnormalities of the striatum and
positron emission tomography and SPECT imaging showed striatal
hypermetabolism and hyperperfusion

Morfopatologie
Caracteristica fazei acute: reacie inflamatorie
exudativ i proliferativ, ce intereseaz esutul
conjunctiv.
Leziunile caracteristice: focare inflamatorii
perivasculare la nivelul vaselor sanguine mici
- diseminate n organism,
- afecteaz n special esutul conjunctiv, cordul, articulaiile
i tegumentele
- faza acut: inflamaie exudativ i proliferativ +
degenerescen fibrinoid a colagenului.

Morfopatologie
1. Leziuni cardiace:

endocard, miocard, pericard


1. nodulii Aschoff miocardici
- leziunea patognomonic a carditei reumatismale, n stadiul

proliferativ

2. miocardita interstiial difuz


3. valvulita verucoas
- inflamaia esutului valvular (cea mai frecvent manifestare clinic
a carditei reumatismale) insuficiene valvulare
- fibroza i calcificarea valvelor stenoze valvulare
- valva aortic i mitral
4. pericardita reumatismal serofibrinoas

Rheumatic Carditis Histology (40X)

Morfopatologie
2. Leziuni extracardiace:
leziuni articulare: inflamaie exudativ sinovial:
vindecare fr sechele
noduli subcutanai
aparat respirator: pleurezie fibrinoas, pneumonie
interstiial
sistem nervos central: infiltrat inflamator perivascular +
degenerescen celular

Tablou clinic
tablou infecios
+
manifestri clinice specifice:
poliartrita,
cardita,
noduli subcutanai,
eritem marginat
chorea minor.

Tablou clinic
Tablou nespecific
febr
altralgii
epistaxis
durere abdominal

2 Patterns of Presentation
Sudden onset = typically begins as polyarthritis
2-6 weeks after streptococcal pharyngitis
Fever
Toxicity

Insidious (subclinical) = initial abnormality is mild


carditis
Age at onset influences complication order:
younger children = carditis, older = arthritis

Tablou clinic
1. Artrita reumatismal
75% - 80%
poliartrit acut migratorie
afectarea predominant a articulaiilor mari
(genunchi, glezna, cot, pumn) i ocazional a celor
mici; foarte dureroase la mobilizare/palpare
cel puin dou articulaii
eroare frecvent: administrarea precoce a
medicaiei antiinflamatoare

Tablou clinic
2. Cardita reumatismal
45-50%
-inflamaie inaparent clinic - miocardit sever

Diagnosticul de cardit reumatismal:


apariia / modificarea unui suflu cardiac organic. Sufluri
semnificative:
suflul sistolic apical de regurgitare mitral
suflul mezo-diastolic Carey-Coombs
suflul diastolic de regurgitare aortic
cordului (Rx i/sau eco)
frectur pericardic / revrsat pericardic - eco
semne de insuficien cardiac congestiv

Tablou clinic

Clasificarea carditei reumatismale:


uoar - sufluri / frectur pericardic; fr
cardiomegalie
medie cardiomegalie, fr manifestri de insuficien
cardiac
sever - insuficien cardiac congestiv

Tablou clinic
3. Nodulii subcutanai
< 5%
dimensiuni reduse, nedureroi, neadereni
localizare:
tendoanele extensorilor,
articulaia pumnului,
cotului,
genunchiului,
la nivelul scapulei i
proceselor spinoase vertebrale
semnificaie: cardit reumatismal sever sau
medie

Tablou clinic
4. Eritemul marginat
<10%
erupie tranzitorie
localizare: n zona proximal a extremitilor
rash-ul este nepruriginos, migrator i poate fi
accentuat sau evideniat prin aplicarea cldurii
locale
semnificaie: cardita reumatismal

Tablou clinic
5. Chorea minor (chorea Sydenham)
(dansul Sfantului Vitus)
15%, femei
perioad de laten: cteva luni de la infecia streptococic.
afectarea sistemului nervos central
tabloul clinic se instaleaz treptat: iniial apare nervozitate,
dificultate munci manuale, mers greoi, mpiedicat; apoi micri
spasmodice, involuntare, necoordonate, haotice ale membrelor,
devin deosebit de violente, + hipotonie muscular; caracter
autolimitat;
se vindec fr sechele n 1 3 luni.

Investigaii paraclinice

Nu exist nici un test de laborator


specific

Investigaii paraclinice
Teste de laborator
- reactanii de faz acut

1. izolarea streptococului de grup A din cultura


faringian (sensibilitate test 25-40%)
2. detectarea anticorpilor antistreptococici:
- ASLO (sensibilitatea testului 80%)(valori patologice: adulti
>240 Todd U; copii >320 Todd U)
- antideoxyribonuclease B (anti-DNAse B), antistreptokinase,
antihyaluronidase, and anti-DNAase (anti-DNPase)

antecedente de infecie streptococcic

Newer
Rapid detection test for D8/17:
This immunofluorescence technique for
identifying the B cell marker D8/17 is
positive in 90% of patients with rheumatic
fever. It may be useful for identifying
patients who are at risk for developing
rheumatic fever.

Investigaii paraclinice

Electrocardiograma
intervalul PR
Ecocardiografia
pancardita reumatismal

Diagnostic pozitiv
criteriile Jones

cel putin dou criterii majore


sau
un criteriu major + dou criterii minore

antecedente de faringit streptococic


Indic o probabilitate crescut pentru diagnosticul de RAA.

Jones Criteria (Revised) for Guidance in the


Diagnosis of Rheumatic Fever*
Major Manifestation
Carditis
Polyarthritis
Chorea
Erythema Marginatum
Subcutaneous Nodules

Minor
Manifestations
Clinical
Previous
rheumatic
fever or
rheumatic
heart disease
Arthralgia
Fever

Laboratory
Acute phase
reactants:
Erythrocyte
sedimentation
rate,
C-reactive
protein,
leukocytosis
Prolonged PR interval

Supporting Evidence
of Streptococal Infection
Increased Titer of AntiStreptococcal 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

Diagnostic pozitiv
Criterii majore

Criterii minore

1. Cardit (cardiomegalie, suflu nou Clinice


aparut, IC, pericardita)
-artralgii (nu e considerat criteriu daca
2. Poliartrit
poliartrita e prezenta)
3. Chorea minor
-febr
4. Eritem marginat
Paraclinice
5. Noduli subcutanai
-reactanilor de faz acut: proteina Creactiv, VSH
- intervalului PR pe ECG

Documentarea faringitei streptococice n antecedente:


-cultura faringian: streptococul beta-hemolitic de grup A
-titrul anticorpi antistreptococici

Diagnostic pozitiv
Sindromul poststreptococic minor
artrita reactiv poststreptococic
artrit i alte manifestri sistemice care apar dup o
faringit streptococic acut
nu se ntrunesc criteriile Jones
nu rspunde dramatic la tratamentul antiinflamator
Importan: unii pacieni pot prezenta ulterior RAA
meninui sub observaie atent cteva luni

Evoluie, prognostic

vindecare n 6-12 sptmni


la 5% din cazuri boala persist peste 6 luni,
ani: cardita reumatismal cronic = tablou
clinic de insuficien cardiac cronic; prognostic
rezervat
recurenele reumatismale - n primii 5 ani

-Rheumatic
heart
disease.
- Abnormal
mitral valve.
- Thick,
fused
chordae

Tratament

nu exist un tratament specific al RAA

Tratament

Obiective majore:
1. tratamentul infeciei streptococice
2. tratamentul atacului acut - Ameliorarea
manifestrilor inflamatorii
3. tratamentul complicaiilor
4. preventie

Tratament

Terapia antibiotic antistreptococic:


Penicilin antibiotic de elecie
doz unic 1,2 milioane U benzatin-penicilin G im.
Pacieni alergici la penicilin : cefalosporine /
Eritromicin 10 zile
profilaxia recurenelor reumatismale

Tratament
Terapia antiinflamatoare
aspirina
nivelul seric eficient: 15-20 mg/dl;
aduli i copii: 80-125 mg/kgcorp/zi
divizare n 4 doze.
se ncepe doar cnd exist diagnostic !!!!
ameliorare simptome + febra doza la 2/3doza de atac pn la
normalizarea datelor de laborator doza la doza de atac, 8
sptmni.
intoleranta la aspirina alte AINS

Corticosteroizi
pacienii cu cardit sever, insuficien cardiac congestiv
prednison: 1-2 mg/kg corp/zi, VSH treptat doza pe o perioad
de 2 sptmni, apoi se ntrerupe. n aceste 2 sptmni: + aspirin,
care se continu 2-3 sptmni dup ntreruperea corticoterapiei.

Tratament
Tratamentul complicaiilor:
Tratamentul insuficienei cardiace:
- repaus la pat
- diuretice
- ? glicozide tonicardiace
Digoxin tahicardia nocturna
! bloc AV
Tratamentul choreei:
- repausul total fizic i psihic + sedare (haloperidol)

Profilaxia RAA

A. Profilaxia primar
B. Profilaxia secundar

Profilaxia primar a RAA

= prevenirea atacului reumatismal iniial prin

antibioterapia corect a infeciei faringiene


produs de streptococul de grup A

Profilaxia primar a RAA


AGENT

DOZ

MOD

DURAT

Benzathine
penicillin G

< 27 kg - 600,000 U
> 27 kg - 1,200,000 U

IM

doz unic

Copii: 250 mg 2-3 times daily


Aduli: 500 mg 2-3 times daily

10 zile

10 zile

SAU

Penicillin V

Pacieni alergici la penicilin:

Erithromicin

40 mg/kg/zi (maxim 1 g/zi)


2-4 ori / zi

Profilaxia secundar a RAA

= prevenirea recurenelor reumatismale

Profilaxia secundar a RAA


AGENT

DOSE

MODE

Benzathine
penicillin G

1,200,000 U
la fiecare 3 4 sptmni

IM

Penicillin V

250 mg x 2 / zi

Sulfadiazine

< 27 kg - 0.5g / zi
> 27 kg - 1.0 g / zi

SAU

Pacieni alergici la penicilin i sulfadiazine:

Cefalopsporine
Erithromicin

250 mg x 2 / zi

Profilaxia secundar a RAA


Durata:
RAA + cardit +
valvulopatie rezidual

10 ani / pn la 40 de ani

RAA + cardit

10 ani / pn la vrst
adult (care e mai lung)

RAA -cardit

5 ani / pn la 21 de ani
(care e mai lung)