A Case of Primary Sjogren's Syndrome with Myocarditis / 대한류마티스학회지

ABSTRACT

Primary Sjogren's syndrome is a chronic autoimmune disorder characterized by xerostomia and keratoconjunctivitis sicca result from lymphocytic infiltrations of salivary and lacrimal glands.It may accompany by the wide spectrums of extraglandular symptoms,such as musculoskeletal,pulmonary,renal,intestinal,hematologic,and nervous system.However,overt cardiac manifestations are very rare and are documented only a few cases sporadically. We report a patient with primary Sjogren's syndrome who developed reversible congestive heart failure as part of a myocarditis.A 39-year-old woman with primary Sjogren's syndrome developed fatigue,dyspnea,and abdominal discomfort.An echocardiography revealed global hypokinetic left ventricle with markedly decreased ejection fraction (EF=39%),abnormal restrictive filling pattern,secondary tricuspid valve insufficiency and no pericardial effusion or thickening.A myocardial biopsy disclosed myocardial degeneration with lymphocyte infiltration.Following treatment with digitalis,diuretics,ACE inhibitor and corticosteroid,the symptoms of congestive heart failure improved.A repeat echocardiography showed an improved ventricular contractility and recovered both diastolic (normalized E/A ratio)and systolic (EF=50%)functions.