Case Presentation: An otherwise healthy 69-year-old woman was admitted to the hospital with a 3-month history of progressive dyspnea on exertion and anasarca following out-patient transthoracic echocardiography (TTE) demonstrating a large pericardial effusion and early signs of cardiac tamponade based on exaggerated mitral inflow velocity with respiration. Physical exam revealed jugular venous distension and a loud systolic murmur with no pericardial rub. ECG showed sinus rhythm with signs of left atrial enlargement and there was prominent cardiomegaly on chest X-ray. Of note, she was recently diagnosed with primary Sjogren’s syndrome (pSS) by parotid gland biopsy and positive antibodies (SS-A and ANA). After diagnosis, she was started on low-dose steroid therapy and trialed on hydroxychloroquine, which was discontinued due to an allergic skin reaction. Pericardiocentesis removed 750 mL of serosanguineous fluid with negative fluid cultures. Cytology showed rare atypical cells of unclear histogenesis without expression of site-specific markers for lung or breast carcinoma. Post-pericardiocentesis, the patient’s symptoms resolved but TTE revealed re-accumulation of a moderate pericardial effusion within 24 hours. One week later, she returned to the emergency department with severe pleuritic chest pain and underwent repeat pericardiocentesis with 600 mL of sanguineous fluid removed. As hemorrhagic pericardial effusion in pSS is very rare, alternative causes including malignancy and tuberculosis were considered. Pericardial fluid acid-fast bacilli and QuantiFERON testing were negative. CT of the chest, abdomen and pelvis revealed an enlarged mediastinal lymph node and multiple thyroid nodules. Thyroid node biopsy was benign and PET-CT scan showed low-level, non-specific uptake in the mediastinal and axillary lymph nodes. She was evaluated by oncology and determined to have no malignant explanation for the recurrent hemorrhagic pericardial effusions. Comprehensive rheumatological testing ruled out mixed connective tissue disorder or systemic lupus erythematosus, which are more commonly associated with pericardial involvement. Sjogren’s syndrome remains the mostly likely etiology of cardiac tamponade in this case. She was treated with a prolonged course of colchicine and corticosteroids, with plan for pericardial window if the effusion recurred.

Discussion: Sjogren’s syndrome is an autoimmune condition with a plethora of extra-glandular manifestations. Cardiovascular involvement is exceedingly rare: there have only been a few reported cases of symptomatic pericardial effusion and one case report of cardiac tamponade associated with pSS. However, studies have shown that TTE detects asymptomatic cardiac involvement in up to 30% of patients. Immunopathology of pericarditis in Sjogren’s syndrome remains unclear; it has been proposed that immune complex deposition in the pericardium triggers an inflammatory response leading to effusion. Therefore, a definitive diagnosis would require identification of specific pericardial histological lesions.

Conclusions: Although rare, physicians must be aware that primary Sjogren’s syndrome may cause pericardial effusions. This case report raises the question of whether all patients with Sjogren’s syndrome would benefit from routine TTE screening. Early identification of pericardial effusions may warrant closer monitoring even in asymptomatic patients to minimize the risk of unexpected cardiac tamponade.