Case Presentation:
A 47-year-old woman with a history of alcohol abuse presented to the emergency department with one week of altered mental status. Review of systems was significant for involuntarily 100 lbs. weight loss over one year with no change in appetite. She reported no prescribed or over-the-counter medication use.
Vital signs on presentation were significant for sinus tachycardia to 115 per minute. She had diffusely thickened and shiny appearing skin with digital ulcers and fingernail pitting. There was bitemporal wasting and bilateral upper and lower limb muscle atrophy. Jugular venous distention was prominent to greater than 15 centimeters H20. S1 and S2 were distant. Pulsus alternans was present. Capillary refill time was greater than 2 seconds.
Complete blood count and complete metabolic panel were unremarkable. EKG demonstrated sinus tachycardia with low voltage. Computed tomography was significant for diffuse emphysematous changes and a pericardial effusion. Echocardiography revealed a large circumferential pericardial effusion with tamponade physiology. Pericardiocentesis revealed an exudative effusion with no evidence for infection or malignancy.
SSA antibody was 7.5 (reference range: 0.0-0.9), C-reactive peptide was 3.0 and anti-nuclear antibody was 7.6 (reference range: 0.0-0.9). Thyroid studies and infectious work-up were negative. A diagnosis of SSc was made based on clinical and laboratory findings.
Forty-three days after initial presentation, follow-up echocardiogram demonstrated recurrence of pericardial effusion with tamponade physiology. A pericardial window was performed and pathology showed chronic fibrous changes. Infectious work-up of pericardial fluid was negative. The patient was started on mycophenolate with improvement in breathing and fatigue level and weight gain.
Discussion:
Scleroderma disorders are hallmarked by thickened, sclerotic skin. Skin thickening with internal organ involvement is the sine qua non of systemic sclerosis (SSc), most commonly affecting the gastrointestinal, pulmonary, and renal systems. Cardiac disease, particularly tamponade is rare.
Pericardial effusion associated with SSc is often asymptomatic and uncovered during autopsy. When present, pericardial effusions are commonly exudative with fibrous changes. Cardiac tamponade associated with SSc is rare. The exact mechanism for tamponade is believed to be due to pericardial fibrosis, restricting pericardial compliance. Tamponade recurrence has been observed in up to 30% of cases. Asymptomatic pericardial disease does not have prognostic significance; however, cardiac tamponade may be a marker for disease severity with estimated 2 and 5-year mortality rates approaching 60 and 75%, respectively.
Conclusions:
We present a rare case of cardiac tamponade associated with SSc. To our knowledge, our case represents one of a handful describing SSc with cardiac tamponade.