This report describes a case of cardiac tamponade as the initial manifestat
ion of unclassified connective tissue disease (UCTD). A 68-year-old Japanes
e woman was admitted to hospital because of dyspnea and edema. She had unde
rgone a radical left mastectomy for the treatment of breast cancer 18 years
before. On admission, bilateral leg edema, hepatomegaly, and a paradoxical
pulse were noted on physical examination. The erythrocyte sedimentation ra
te was elevated and the C-reactive protein was 2.8 mg/dl. Antinuclear antib
odies and anti-SS-A/Ro antibodies were present. The scl-70 and anticentrome
re antibodies were elevated. Chest radiography showed cardiomegaly. Echocar
diography revealed a large pericardial effusion, but the pericardial fluid
did not contain malignant cells or bacteria. She did not meet the diagnosti
c criteria for any known connective tissue diseases, so was diagnosed with
cardiac tamponade due to UCTD. Prednisolone (30 mg/day) was administered, w
hich resulted in a gradual resolution of the pericardial effusion. Although
connective tissue diseases are known to cause pericardial effusion, cardia
c tamponade as the initial manifestation of the disease in the absence of o
ther symptoms is quite rare.