Cardiac involvement in systemic lupus erythematosus (SLE) is very common. Despite the relative frequency of pericarditis and pericardial effusions which occur in more than 50% of SLE patients, cardiac tamponade is rare and is seen in less than 3%. Additionally, there have been no consistent reliable predictors of tamponade development in such patients. We report a case of tamponade as an isolated symptomatic event in a patient with known SLE.
Case Presentation: A 39-year-old female patient with SLE presented with a one week history of progressive dyspnea, pleuritic left-sided chest pain, malaise, nausea and vomiting, but no fever or arthralgia. The pain worsened on lying flat but improved with sitting upright. Physical exam was relatively unrevealing with no distant heart sounds, pericardial rub, elevated JVP or pulsus alternans. CXR showed cardiomegaly and EKG showed sinus tachycardia with low voltage but was otherwise unremarkable, specifically lacking electrical alternans. An elevated D-dimer coupled with her symptoms prompted a chest CT scan which was negative for pulmonary embolism but revealed a large pericardial effusion. A stat echocardiogram showed a moderate-to-large circumferential pericardial effusion with M-mode and Doppler findings suggestive of early tamponade physiology. Serological markers again confirmed SLE and she notably had low C4 complement levels. Pericardiocentesis was performed with intra-pericardial instillation of triamcinolone to prevent recurrence. High-dose steroids and colchicine, as is indicated in the treatment of lupus-associated effusions and pericarditis, were also administered and her chest pain and dyspnea dramatically improved. Repeat ECHO showed interval decrease in effusion size, and one month later showed complete resolution.
Discussion: Pericarditis and effusions are common in SLE; however, tamponade is rare with an incidence of 1-3%. Very few studies have identified clinical predictors of developing tamponade. Moreover, there is no evidence to support the use of these guidelines in clinical practice. Only one paper exists which suggests that a low C4 level is predictive of tamponade development. In our patient, the identification of tamponade was somewhat incidental, as her initial work-up was not wholly suggestive. Her low C4 level could have possibly been of diagnostic value had this been determined prior.
Conclusions: Although cardiac tamponade is a rarity in Lupus patients, the possibility of this life-threatening event should be considered particularly when the symptomatology or hemodynamic status of the patient is suggestive. There are no guidelines which suggest screening for pericardial effusions in this population given its relative frequency, and moreover no clinical predictors of tamponade development have been established. However, careful consideration may be given to patients with low C4 levels, although the clinical significance and practicality of this remains to be elucidated.
