Background/Purpose: Although cardiac involvement is symptomatic in only 5% of patients with sarcoidosis, it is considered an important prognostic factor and the second leading cause of death in patients with sarcoidosis.  There are no formal guidelines for the diagnosis or management of cardiac sarcoidosis (CS). Due to the rarity of this disease, prospective clinical trials are lacking and difficult to conduct.  Corticosteroids are the mainstay of treatment in CS. Steroid sparing agents mainly methotrexate, azathioprine, mycophenolate mofetil and TNF-alpha inhibitors (Tumor Necrosis Factor).  Our aim is to report successful treatment of CS with a combination therapy consisting of a TNF-alpha inhibitors and an oral immunosuppressive agent.

Methods: This is a retrospective chart review of patients with sarcoidosis who were evaluated either by a rheumatologist or a pulmonologist at a tertiary academic medical center between January 1^st2013 and December 31st 2014.  Patients with biopsy proven sarcoidosis (with the exception of endomyocardial biopsy) were included.  Presence of hypermetabolic activity on the cardiac fluorodeoxyglucose (FDG) positron emission tomography (PET) scan was used to establish the diagnosis of CS. PET scan was repeated after 6-12 months to assess response to therapy.

Results: The charts of 103 patients with a diagnosis of sarcoidosis were reviewed and only 81 patients with biopsy proven sarcoidosis were included. Fifteen out of 81 patients (18.5%) had cardiac FDG PET suggestive of CS. All cases had other manifestations of systemic sarcoidosis, with lymphadenopathy and pulmonary parenchymal involvement as the most common affected areas. Clinical manifestations of CS included chest pain with or without elevation of cardiac enzymes, a reduced ejection fraction, palpitations and conduction abnormalities on ECG. Out of 15 patients, 9 patients were treated with a combination regimen of TNF-alpha inhibitors (infliximab in 8 patients, adalimumab in 1 patient) and an oral immunosuppressive agent (mycophenolate mofetil in 7 patients, azathioprine in 2 patients). Prednisone was also used in all but one patient, with doses ranging from 10 mg to 60mg daily. Six patients were not included in our study as they were eventually managed elsewhere and immunosuppressive treatment was withheld in one patient due to pregnancy. A repeat Cardiac FDG PET scan after 6-12 months of therapy showed a marked improvement or resolution of the hypermetabolic activity in all 9 patients. Cardiac and extracardiac manifestations improved or resolved following treatment.

Conclusion: We describe the clinical presentation and treatment outcomes of 9 patients with cardiac sarcoidosis successfully managed with a combination of a TNF inhibitor and an oral immunosuppressive agent. To our knowledge, this is the largest series of cardiac sarcoidosis successfully treated with such a combination therapy.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/successful-treatment-of-cardiac-sarcoidosis-with-biologic-and-immunosuppressive-combination-therapy/