Cardiac tamponade as an initial manifestation of
undifferentiated connective tissue diseases (UCTD) is extremely rare, with only one case reported in literature thus far. We describe here, a case of a middle-aged man who presented with symptoms of
fatigue, exertional
dyspnea and
orthopnea. His physical exam was significant for
anasarca, elevated JVP and pulsus paradoxus. Chest X-ray showed
pleural effusions and
cardiomegaly, electrocardiogram revealed electrical alternans and a transthoracic echocardiogram demonstrated massive
pericardial effusion with hemodynamic compromise. There was clear evidence of tamponade on right heart catheterization. All common causes of
pericardial effusion were assiduously excluded before working up the patient for connective tissue disorders, which revealed a high
antinuclear antibody titer (1:160), grossly elevated SSA, SSB
antibodies and increased
C-reactive protein levels (13.04 mg/dl). Patient had no signs or symptoms suggestive of
systemic sclerosis (
xerophthalmia or
xerostomia) and did not meet criteria for any other known
connective tissue diseases. He was therefore diagnosed with UCTD, and successfully treated with
colchicine after emergency pericardiocentesis. This case presents UCTD as a rare cause of
cardiac tamponade and large
pericardial effusions and suggests that
colchicine can be used to treat UCTD-associated effusions. These patients once diagnosed, are at risk of developing known
connective tissue diseases within 5 years of disease onset and should be followed up in clinic periodically.