IgG4-Related Systemic Disease Presenting with Pleural Effusion, Ascites and Dilated Cardiomyopathy

Purpose We report the case of a patient with IgG4-related disease with multiple organ involvement, especially myocardium, that was successfully treated with prednisone. Methods We performed several serological tests, electrocardiogram, echocardiography, computed tomography and inguinal lymph node biopsy. Results We diagnosed the patient with IgG4-related disease by the elevated serum IgG4 level and histological lymph node findings. Conclusions This is the first reported case of IgG4-related disease with dilated cardiomyopathy.