Malattia reumatica acuta

Transcription

Malattia reumatica acuta
Infezione e malattie reumatiche
Meccanismo del danno
Infezione causa diretta di
infiammazione del tessuto
Prototipi
Artrite da piogeni
Condizioni predisponenti
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Generali:
-diabete
-deficit imm.
Locali:
-danni strutturali
Infezione e malattie reumatiche
Meccanismo del danno
Organismi intatti (±)
Cross-reattività con Ag ‘self’
Prototipi
Artriti reattive
Condizioni predisponenti
MHC I classe
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Artrite reattiva
Artrite acuta, non suppurativa,
sterile che si sviluppa dopo
2-4 settimane da un’infezione
batterica occorsa in una sede
distante dall’articolazione.
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Artrite post-infettiva
Artrite acuta, non suppurativa,
che si sviluppa dopo 2-4
settimane da un’infezione
batterica extra-articolare.
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Microbial Infections Associated with Reactive Arthritis
Enteric Bacteria
• Salmonella: various serovars
• Shigella
S. Flexneri
S. Dysenteriae
S. Sonnei
• Yersinia
Y. enterocolitica (especially O:3 and O:9)
Y. Pseudotuberculosis
•  Campylobacter
C. Jejuni
C. Coli
Clostridium difficile
Bacteria Causing Urethritis
•  Chlamydia trachomatis
Mycoplasma genitalium∗
Ureaplasma urealyticum∗
Bacteria Causing Upper Respiratory Infection
Beta-hemolytic streptococcus∗
Chlamydia pneumoniae
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Evolving concept of "reactive
arthritis"
Arthritis induced by bacterial infections in
which live bacteria cannot be detected in the
affected joints
Arthritis induced by the following bacteria:
Chlamydia, Salmonella, Shigella, Yersinia, and
Campylobacter
The wide variety of cross-reactions between streptoccoccal
antigens and mammalian tissues
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Malattia reumatica acuta
Reumatismo articolare acuto
Febbre reumatica
Malattia di Bouillaud
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Malattia reumatica acuta
Sequela ritardata, non
suppurativa, di infezione
dell’orofaringe da
streptococco β-emolitico di
gruppo A.
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Reported Rheumatic Fever Incidence in Denmark
1862-1962
1928 Fleming scopre la penicillina
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Acute Rheumatic Fever
Only a few M serotypes (types 5,
14, 18, 24) have been identified
with outbreaks of ARF, suggesting
that certain strains of group A
streptococci
may
be
more
"rheumatogenic" than others.
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Evidence implicating the group
A streptococcus
Outbreaks of acute rheumatic
fever closely follow epidemics of
either streptococcal sore
throats or scarlet fever.
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Evidence implicating the group
A streptococcus
Adequate treatment of a
documented streptococcal
pharyngitis markedly reduces
the incidence of subsequent
acute rheumatic fever.
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Evidence implicating the group
A streptococcus
Appropriate antimicrobial
prophylaxis prevents the
recurrences of disease in known
patients with acute rheumatic
fever.
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Evidence implicating the group
A streptococcus
If the sera of the majority of acute
rheumatic fever patients is tested for
three antistreptococcal antibodies
(streptolysin "O", hyaluronidase, and
streptokinase), the vast majority of the
patients (whether or not they recall an
antecedent streptococcal sore throat)
will have elevated antibody titers to
these antigens.
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Williams RC Jr. Am J Med. 1983
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Acute Rheumatic Fever
An acute systemic inflammatory illness which
usually occurs 2-4 weeks after group A ßhemolytic streptococcal infection of the
pharynx.
 
It appears to be related to a series of
immunologic reactions to antigenic components
of the streptococcus, which also cross-react
with various human tissues, including heart
muscle, valvular structures, articular tissues and
neuronal antigens.
 
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Acute Rheumatic Fever
(Malattia Reumatica Acuta)
Acute febrile illness:
  The patient may present with migratory
arthritis predominantly involving the large
joints of the body.
  2) There may be concomitant clinical and
strumental signs of carditis and valvulitis.
  3) There may be involvement of the central
nervous system (CNS), manifesting itself as
Sydenham's chorea.
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The many faces of acute reumatic fever:
possible features
•  High fever, prostration, crippling polyarthritis
•  Lassitude, tachycardia, new cardiac murmurs
•  Acute pericarditis
•  Fulminant heart failure
•  Sydenham’s chorea without fever
•  Acute abdominal pain mimicking appendicitis
•  Varying combinations of the above
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Rheumatic fever
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Rheumatic fever
Carditis of acute rheumatic
fever
Pancarditis
Involving the pericardium,
myocardium, and endocardium.
Between 40 and 60% of patients
with ARF have evidence of carditis.
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Carditis of acute rheumatic fever
One or more of the following:
  sinus tachycardia
  murmurs
  S3 gallop
  pericardial friction rub
  cardiomegaly
  congestive heart failure
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Murmurs in carditis of acute reumatic
fever
  apical pansystolic (mitral regurgitation)
  apical mid-diastolic (mitral valvulitis)
(Carey Coombs murmur)
  basal diastolic (aortic regurgitation)
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Erythema marginatum in a child with acute
rheumatic fever
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Erythema nodosum
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Histologic picture of a rheumatic fever nodule
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Criteri di Jones
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Criteri diagnostici vs criteri
classificativi
•  Diagnostici: utili per la diagnosi nel singolo
paziente (comprendono manifestazioni
frequenti anche se aspecifiche)
•  Classificativi: utili per assicurare la
comparabilità fra casistiche raccolte in
centri
diversi
(si
fondano
su
manifestazioni specifiche)
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Treatment of Acute Rheumatic Fever
Anti-inflammatory treatment Mild or no carditis: Aspirin 50–100 mg/kg/d
in 4 divided doses for 2–4 weeks, then taper
over 4–6 weeks.
Moderate or severe carditis: prednisone 2
mg/kg/d in 2 doses for 2–4 weeks, then
taper with addition of aspirin when
prednisone dose is <0.5 mg/kg/d
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Primary anti-streptococcal therapy
1.2 million units of benzathine
penicillin G i.m. or oral penicillin or
erythromycin for 10 days
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Secondary prevention of rheumatic fever
(prevention of recurrent attacks)
AGENT
Benzathine penicillin G
DOSE
1.200.000 U every 4 wk
MODE
Intramuscular
or
Penicillin V
250 mg twice daily
Oral
or
Sulfadiazine
0.5 g once daily for patients
≤ 27 Kg
1,0 g once daily for patients
Oral
> 27 Kg
For individuals allergic to Penicillin and Sulfadiazine
Erytrhomycin
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250 mg twice daily
Oral
Recommendations for Duration of Antimicrobial
Prophylaxis in Patients with Acute Rheumatic Fever
Patients with rheumatic fever with carditis and
residual heart disease
  At least 10 years after the last episode and at
least until age 40, sometimes lifelong prophylaxis
Rheumatic fever with carditis but no residual
heart disease
  10 years or well into adulthood, whichever is
longer
Rheumatic fever without carditis
  5 years or until age 21 years, whichever is
longer
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Post-streptococcal Arthritis
 
The latent period between the antecedent
streptococcal infection and the onset of reactive
arthritis is shorter (1 to 2 weeks) than the 3 to 4
weeks usually seen in classic acute rheumatic fever.
  The response to aspirin and other NSAIDs is poor in
comparison to the dramatic response seen in classic
rheumatic fever.
 
Evidence of carditis is not usually seen in these
patients; further, the severity of the arthritis is
quite marked.
 
Extra-articular manifestations (such as tenosynovitis
and renal abnormalities) are often seen in these
patients.
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