A 46 year-old woman presented with one week of palpitations and dyspnea on exertion. Her medical history was remarkable for a pericardial effusion of unknown etiology two months prior, treated with pericardiocentesis in Guyana. On presentation she was hemodynamically stable. Exam was significant for distant heart sounds and decreased breath sounds at the lung bases. Erythrocyte sedimentation rate was 50 mm/hr and c-reactive protein was 214 mg/L. Antinuclear antibodies were elevated (1:640 homogenous), with normal rheumatoid factor, antibodies to double-stranded DNA, antinuclear RNP, anti-Smith, anti-Ro, and anti-La. All culture data including tuberculosis and fungal cultures were negative. Electrocardiogram was significant for low voltage without electrical alternans. Chest computed tomography showed a large nonhemorrhagic pericardial effusion causing mass effect upon the right ventricle, as well as a moderate sized left pleural effusion. No hilar adenopathy or lung nodules were seen. Transthoracic echocardiography revealed a large, mostly organized, echogenic pericardial effusion with normal left ventricular ejection fraction. The patient underwent a pericardial window and greater than one liter of serosanginous fluid was removed. Pericardial biopsy revealed non-necrotizing granulomas and mesothelial cells with chronic inflammation, highly suggestive of cardiac sarcoidosis. Initiation of daily prednisone and weekly methotrexate were recommended, with plan for cardiac magnetic resonance imaging.
Discussion:
Though most commonly manifesting in the lungs, sarcoidosis is a systemic disease that generally involves multiple organs. Interestingly, the concept of isolated cardiac sarcoidosis with no additional organ involvement was first introduced in the literature in 2001 by a Japanese group [1]. While cardiac sarcoidosis can present as an arrhythmia, heart failure, or pericardial disease, pericardial effusion has been mostly reported as asymptomatic trace to small effusions. There are limited case reports of sarcoidosis presenting with massive pericardial effusion. Of those, only a few documented massive and recurrent pericardial effusion as the sole clinical presentation of sarcoidosis [2, 3].
The diagnosis of cardiac sarcoidosis is challenging. Various guidelines have been proposed requiring clinical, radiographic, and pathological evidence. Our patient had clinical features, positive inflammatory markers, and non-caseating granulomas on pericardial biopsy. While optimal management for cardiac sarcoidosis is uncertain, corticosteroids are still the mainstay of therapy.
Conclusions:
Sarcoidosis can present as a massive recurrent pericardial effusion in the absence of other systemic findings consistent with sarcoid. It is important for clinicians to consider sarcoidosis in the differential diagnosis of isolated recurrent pericardial effusion and to consider starting steroid treatment early to prevent complications.