Background/Purpose: Sarcoidosis is a multisystem granulomatous disease of unclear etiology characterized histologically by non-caseating granulomas. While clinically manifest cardiac involvement occurs in only about 5% of patients with sarcoidosis, a significant proportion have clinically silent disease. Symptomatic cardiac involvement portends a poorer prognosis with manifestations varying from heart failure and conduction abnormalities to ventricular arrhythmias including sudden death. Immunosuppression with corticosteroids and DMARDs has been the mainstay of treatment despite a paucity of data. There is a subset of patients that are either non-responders to these agents or in whom the side effect profile is prohibitive for their long-term use. Biologic agents, mainly TNF alpha antagonists, have been used as salvage therapies in these patients. However, the evidence regarding their efficacy and safety is limited to a few case reports. In fact, there remains much apprehension regarding the use of TNF alpha antagonists in patients with systolic heart failure due to concerns that they can exacerbate heart failure. Our objective was to study the efficacy and safety of using biologics for the treatment of cardiac sarcoidosis.

Methods: We conducted a retrospective and prospective observational study of all adult patients with cardiac sarcoidosis treated with biologics at an academic medical center in Washington D.C, USA between 2013 and 2018.

Results: We identified 9 patients (3 men and 6 women) diagnosed with cardiac sarcoidosis at our institution. The mean age at diagnosis was 49.9 ± 8.6. 1 patient was Caucasian and the rest (n=8) were African American. Lungs were the most common extra cardiac organ involved (n=7) followed by CNS (n=4), liver (n=4) and skin (n=3). 5 of the patients presented with systolic heart failure (EF< 50%), 3 with arrhythmias and 1 was found to have incidental abnormal myocardial uptake on PET imaging. 8 of the 9 patients had abnormal myocardial uptake on PET imaging. All patients were initially treated with oral steroids and 7 of the 9 patients also received oral DMARDs; MTX (n=6), AZA (n=2), HCQ (n=2) and MMF (n=1). Biologics used were adalimumab (n=5), infliximab (n=3) and rituximab (n=1). The most common indication for biologics was progression of disease despite optimal doses of standard therapy, followed by intolerance or contraindication to standard therapy. 75% of the patients were noted to have marked clinical improvement with the addition of a biologic. 5 out of 9 patients had decreased myocardial uptake on PET following treatment with a biologic. 1 patient had no change on PET and 3 have not had repeat imaging done yet. None of the patients had worsening of left ventricular systolic function with the addition of a TNF alpha antagonist. There were no reported major infections or significant adverse events that were attributable to the use of biologics.

Conclusion: Based on our small cohort, biologics (mainly TNF alpha antagonists) appear to be safe and efficacious as salvage therapy for cardiac sarcoidosis. However, there is a need for prospective studies to further validate these findings as well as to identify the subset of patients that would benefit from early initiation of these therapies.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/experience-with-biologic-agents-for-the-treatment-of-cardiac-sarcoidosis-in-a-u-s-academic-medical-center/