Case Presentation: A 43-year-old woman presented to the emergency department for sudden-onset of right calf pain, dyspnea, and coughing of blood-tinged sputum, along with worsening of right-sided, dull, heavy, pleuritic chest pain which had otherwise been present for three weeks. She was diagnosed with systemic lupus erythematosus four days prior to presentation. On physical exam, pulse rate was 114/min and respiration rate was 18/min. Cardiopulmonary examination revealed crackles at the base of the lungs and a pericardial friction rub. Electrocardiogram showed sinus tachycardia and new right-axis deviation. Computed tomography pulmonary angiography (CTPA) revealed an acute pulmonary embolus involving the distal right main pulmonary artery that extended into the lobar branches. Transthoracic echocardiography showed a dilated right ventricle with severely reduced systolic function, tricuspid valve regurgitation, and a large circumferential pericardial effusion. The central venous pressure was elevated, further supporting the presence of right heart strain. A continuous heparin infusion was started. The following day, she underwent a right heart catheterization, which confirmed pulmonary hypertension, and a diagnostic pericardiocentesis. The pericardial effusion was not completely drained due to fear that doing so would leave the effects of the pulmonary embolism on the right ventricle unopposed, leading to worsening right ventricular dysfunction. Pericardial fluid analysis revealed a non-bloody effusion. She subsequently underwent ultrasound-assisted catheter-directed thrombolysis. Over the next several days, the pericardial effusion was drained in small increments, with the amount and frequency of fluid removed guided by serial echocardiographic studies. Unfortunately, the pericardial effusion reaccumulated. Ultimately, she required a video-assisted thoracoscopic pericardiectomy. She was discharged from the hospital one week later.
Discussion: Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect any organ in the body. Although SLE is commonly associated with both pulmonary emboli and pericardial effusions, their co-occurrence in this patient population is rare. This case is especially unique in that the presence of each of these two opposing disease processes dictated the management of the other. Thrombolytic therapy for the pulmonary embolus could not be implemented until a hemopericardium had been excluded via pericardial fluid analysis. Similarly, the pericardial effusion was not completely drained during the initial pericardiocentesis due to the risk of precipitating obstructive shock by leaving the effects of the pulmonary embolism on the right ventricle unopposed. This case report underscores a complex patient with SLE whose management required careful consideration and balancing of cardiovascular hemodynamics.
Conclusions: Systemic lupus erythematosus is a multisystem disease with a myriad of clinical presentations and combinations thereof. Providers caring for patients with SLE should maintain a high index of suspicion for the various possible manifestations of the disease, remembering that the presence of one does not preclude that of others. When manifestations co-exist, management should be individualized based on an understanding of the physiologic interactions of each conflicting pathology.