PurposeThere is currently little evidence to support intravenous immune globulin (IVIG) therapy for pediatric myocarditis. The purpose of our retrospective study was to assess the effects of IVIG therapy in patients with presumed myocarditis on survival and recovery of ventricular function and to determine the factors associated with its poor outcome.MethodsWe reviewed all consecutive cases of patients with myocarditis with left ventricular dysfunction verified by echocardiogram who had visited 3 university hospitals between January 2000 and September 2009. These patients were divided into 2 groups. Group 1 consisted of 23 patients (69.6%) who received IVIG alone or IVIG in combination with steroids, and group 2 consisted of 10 patients (30.3%) who received neither IVIG nor other immunosuppressive agents. Clinical manifestations, laboratory results, echocardiographic findings, and outcomes were compared between these 2 groups.ResultsOne year after the initial presentation, the difference in the probability of survival did not show statistical significance in IVIG-treated patients (P=0.607). Of the echocardiographic parameters on admission, a shortening fraction of less than 15% was associated with unremitting cardiac failure. Furthermore, anemic patients were more likely to have elevated N-terminal fragment levels of the B-type natriuretic peptide (NT-proBNP) in the progressed group (P=0.036).ConclusionThere was no difference between the IVIG-treated patients and the control patients in the degree of recovery of left ventricular function and survival. Prospective, randomized, clinical studies are needed to elucidate the effects of IVIG treatment during the acute stage of myocarditis on ultimate outcomes.

Rheumatic fever is an acute inflammatory sequela following a group A, β-hemolytic streptococcal infection. Rheumatic fever is characterized by polyarthritis, carditis, chorea, subcutaneous nodules, and erythema marginatum as the major diagnostic criteria. Rarely, advanced heart block may also occur. A 13-year-old boy was admitted to the Pediatric Department for evaluation and management of complete atrioventricular block. The patient had exertional dyspnea for 1 month. Based on the findings of mitral regurgitation, fever, elevated acute phase reactants, and a high antistreptolysin O titer, the patient was diagnosed with rheumatic fever. A benzathine penicillin injection was administered, as well as salicylate therapy. On the 5th day of hospitalization, the electrocardiogram revealed a normal sinus rhythm with a 1st degree atrioventricular block. After discharge, the electrocardiogram normalized with the monthly penicillin injections. Herein we report a case of complete atrioventricular block associated with rheumatic fever. The heart block resolved without specific cardiac treatment, other than a non-steroidal anti-inflammatory medication.

Because of the various body sizes of children with Kawasaki disease (KD), coronary artery diameter requires normalization to the body surface area as a z score.In updated guidelines, coronary artery abnormalities are important criteria in the diagnosis of KD, and z score systems have been accepted to define coronary artery abnormalities.However, the z score formula should be selected carefully because each yields different results.

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Neonatal lupus is a rare rheumatic disease. Clinical manifestations include characteristic annular or macular rashes, congenital heart block, cytopenias, and hepatitis. Neonatal lupus is caused by transmission of maternal immunoglobulin G autoantibodies such as anti-SSA/Ro antibody or anti-SSB/La antibody to the fetus through the placenta. We report two cases of neonatal lupus. The first case refers to an 18-day-old male with annular rashes on both cheeks, neutropenia, positive tests for antinuclear antibody, anti-SSA/ Ro antibody, and anti-SSB/La antibody. His mother was diagnosed with systemic lupus erythematosus characterized by positive tests for antinuclear antibody, anti-SSA/Ro antibody, and anti-SSB/La antibody. The second case represents a 32-day-old female with annular rash on both hands, soles, and the genital area, neutropenia, hepatitis, positive tests for antinuclear antibody, and anti-SSA/Ro antibody. Skin punch biopsy was conducted. Her mother did not have history of connective tissue diseases. We referred her mother to the division of rheumatology of the department of internal medicine. The mother was suspected with primary Sjögren's syndrome because of arthralgia and dry eye symptoms with positive tests for antinuclear antibody, anti-SSA/Ro antibody, anti-SSB/ La antibody, and rheumatoid factor. It is necessary to suspect neonatal lupus in neonates or infants with characteristic annular rash with or without maternal history of connective tissue disorders.