Cardiac sarcoidosis with a twist - active and fibrotic sarcoid with antiphospholipid positivity: A case report

Cardiac sarcoidosis (CS) is an infiltrative disease with manifestations such as non-sustained ventricular tachycardia (NSVT) and heart failure (HF). Antiphospholipid syndrome (APS) and antiphospholipid positivity (APP) are prothrombotic phenomena which elevate risk for thromboembolism. CS with active systemic sarcoid and APS/APP is a rare combination of diseases.

A 54 year old male with HF presented with several cardiopulmonary symptoms. Chest imaging showed bilateral patchy and reticulonodular infiltrates. Subsequent lung biopsy confirmed pulmonary sarcoidosis. Positron emission tomography revealed active systemic sarcoidosis (SS) and fibrotic CS. Positive antiphospholipid antibodies without thromboembolism confirmed APP. HF and APP were managed with medical therapy. Fibrotic CS and NSVT required permanent cardiac device and antiarrhythmic therapy. SS was managed with early taper of steroids and transition to biologics.

Fibrotic CS with active SS and APS/APP has not been previously described in literature. This case utilized a modified approach for the management of this combination of diseases. As immunosuppressants such as steroids have limited utility in fibrotic sarcoidosis and a potential for thromboembolic complications in the presence of APP, an accelerated transition to non-thrombotic immunosuppressants can be advantageous in the long term treatment of this combination of diseases.