Fig. 11.19 Rheumaticheartdisease.(A)Acuterheumaticmitralvalvulitissuperimposedonchronicrheumaticheartdisease.Smallvegetations(verrucae)arevisiblealongthelineofclosureofthemitralvalveleaflet(arrows). Previousepisodesofrheumaticvalvulitishavecausedfibrousthickeningandfusionofthechordaetendineae.(B)MicroscopicappearanceofanAschoffbodyinacuterheumaticcarditis;thereiscentralnecrosisassociatedwithacircumscribedcollectionofmononuclearinflammatorycells,includingsomeactivatedmacrophageswithprominentnucleoliandcentralwavy(caterpillar)chromatin(arrows). (CandD)Mitralstenosiswithdiffusefibrousthickeninganddistortionofthevalveleaflets,commissuralfusion(arrows), andthickeningandshorteningofthechordaetendineae.Thereismarkedleftatrialdilationasseenfromabovethevalve(C).(D)Anteriorleafletofanopenedrheumaticmitralvalve;notetheneovascularization(arrow).(E)Surgicallyremovedspecimenofrheumaticaorticstenosis,demonstratingthickeninganddistortionofthecuspswithcommissuralfusion.(E, From Schoen FJ, St John-Sutton M: Contemporary issues in the pathology of valvular heart disease, HumPathol18:568, 1967.) about 20% of first attacks occur in adults, with arthritis being the predominant feature. Symptoms in all age groups typically begin 2 to 3 weeks after streptococcal infection and are heralded by fever and migratory polyarthritis— one large joint after another becomes painful and swollen for a period of days, followed by spontaneous resolution with no residual disability. Although cultures are negative for streptococci at the time of symptom onset, serum titers of antibodies against one or more streptococcal antigens (e.g., streptolysin O or DNAase) usually are elevated. The clinical signs of carditis include pericardial friction rubs and arrhythmias; myocarditis may be sufficiently severe to cause cardiac dilation and resultant functional mitral insufficiency and CHF. Nevertheless, less than 1% of patients die of acute rheumatic fever.
The diagnosis of acute rheumatic fever is made based on serologic evidence of previous streptococcal infection in conjunction with two or more of the Jones criteria: (1) carditis; (2) migratory polyarthritis of large joints; (3) subcutaneous nodules; (4) erythematous annular rash (erythema marginatum) in the skin; and (5) Sydenham chorea, a neurologic disorder characterized by involuntary purposeless, rapid movements (also called St. Vitus dance). Minor criteria such as fever, arthralgias, EKG changes, or elevated acute phase reactants also can help support the diagnosis.