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 scaricato da www.sunhope.it 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 scaricato da www.sunhope.it 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. scaricato da www.sunhope.it Artrite post-infettiva Artrite acuta, non suppurativa, che si sviluppa dopo 2-4 settimane da un’infezione batterica extra-articolare. scaricato da www.sunhope.it 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 scaricato da www.sunhope.it 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 scaricato da www.sunhope.it scaricato da www.sunhope.it Malattia reumatica acuta Reumatismo articolare acuto Febbre reumatica Malattia di Bouillaud scaricato da www.sunhope.it Malattia reumatica acuta Sequela ritardata, non suppurativa, di infezione dell’orofaringe da streptococco β-emolitico di gruppo A. scaricato da www.sunhope.it Reported Rheumatic Fever Incidence in Denmark 1862-1962 1928 Fleming scopre la penicillina scaricato da www.sunhope.it 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. scaricato da www.sunhope.it Evidence implicating the group A streptococcus Outbreaks of acute rheumatic fever closely follow epidemics of either streptococcal sore throats or scarlet fever. scaricato da www.sunhope.it Evidence implicating the group A streptococcus Adequate treatment of a documented streptococcal pharyngitis markedly reduces the incidence of subsequent acute rheumatic fever. scaricato da www.sunhope.it Evidence implicating the group A streptococcus Appropriate antimicrobial prophylaxis prevents the recurrences of disease in known patients with acute rheumatic fever. scaricato da www.sunhope.it 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. scaricato da www.sunhope.it Williams RC Jr. Am J Med. 1983 scaricato da www.sunhope.it 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. scaricato da www.sunhope.it 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. scaricato da www.sunhope.it 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 scaricato da www.sunhope.it Rheumatic fever scaricato da www.sunhope.it 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. scaricato da www.sunhope.it scaricato da www.sunhope.it Carditis of acute rheumatic fever One or more of the following: sinus tachycardia murmurs S3 gallop pericardial friction rub cardiomegaly congestive heart failure scaricato da www.sunhope.it Murmurs in carditis of acute reumatic fever apical pansystolic (mitral regurgitation) apical mid-diastolic (mitral valvulitis) (Carey Coombs murmur) basal diastolic (aortic regurgitation) scaricato da www.sunhope.it Erythema marginatum in a child with acute rheumatic fever scaricato da www.sunhope.it Erythema nodosum scaricato da www.sunhope.it Histologic picture of a rheumatic fever nodule scaricato da www.sunhope.it scaricato da www.sunhope.it scaricato da www.sunhope.it Criteri di Jones scaricato da www.sunhope.it 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) scaricato da www.sunhope.it 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 scaricato da www.sunhope.it Primary anti-streptococcal therapy 1.2 million units of benzathine penicillin G i.m. or oral penicillin or erythromycin for 10 days scaricato da www.sunhope.it 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 scaricato da www.sunhope.it 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 scaricato da www.sunhope.it 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. scaricato da www.sunhope.it scaricato da www.sunhope.it