Snapshot A 13-year-old-boy presents with 3 days of malaise, painful joints, nodular swelling over his elbows, and a low-grade fever. Two weeks ago he was seen by his pediatrician for a sore throat. You observe a well-defined rash on his trunk and shoulders. Introduction An autoimmune disease sequela of untreated pharyngeal streptococcal infection Caused by cross-reactions between strep antigens and antigens on joint and heart tissue Systemic immune process that may or may not lead to RHD Risks include right-sided heart disease secondary to valvular dysfunction most often mitral stenosis Presentation Symptoms usually present approximately two weeks following Streptococcal pharyngitis joint pain rash skin nodules neurlogic dysfunction (motor instability) fever Physical exam diagnosed using the Jones criteria evidence of a preceding group A streptococcal infection plus two major critertia or one major and two minor criteria Major criteria (PECCS) Minor criteria Joints - arthritis <3 Carditis (myocarditis, pericarditis) Nodules - subcutaneous Erythema marginatum Syndenham's chorea Fever Antecedent strep infection Athralgias Acute phase reactions elevated ESR and/or CRP Prolonged PR interval Evaluation Diagnosis is based primarily on clinical observation (Jones' criteria) and history Labs positive ASO (reflects past immunologic event) elevated ESR and/or CRP Rapid antigen detection test (RADT) +/- throat culture EKG may show evidence of RHD secondary to mitral valve involvement Echo Differential RA, SLE, endocarditis, osteomyelitis, Lyme's Disease, sickle cell disease Treatment Medical management amoxicillin penicillin indicated for acute rhuematic fever azithromycin in penicillin-allergic patients Prognosis, Prevention, and Complications Prognosis good to very good if diagnosed and treated aggressively antibiotic prophylaxis is key to maintaining good outcomes Prevention primary prophylaxis: prompt treatment of GAS infection secondary prophylaxis: after rheumatic fever (if no carditis) until age of 21 or 5 years to reduce recurrent episodes and subsequent complications Complications RHD secondary to autoimmune response affecting mitral valve