Background/Purpose: CS is a life-threatening condition that accounts for 85% of sarcoidosis-related deaths in Japan and 13-25% in America. The objective is to evaluate the effectiveness and tolerance of TNFI treatment in CS.

Methods: From a French multicenter cohort of patients with extra-thoracic sarcoidosis, we retrospectively analyzed patients who fulfilled following inclusion criteria 1) a definite histologically proven extra-thoracic sarcoidosis, 2) a CS based on the 2011-Heart Rhythm Society consensus and 3) who received a TNFI. The response to TNFI treatment was analyzed on multiple criteria including (i) cardiac clinical symptoms/signs i.e. New York Heart Association (NYHA) class for dyspnea, heart failure, and cardiac rhythm or conduction disturbances; (ii) NT-pro-BNP and BNP serum levels, and (iii) cardiac imaging abnormalities on echography, scintigraphy, MRI, or 18 FDG PETscan. Patients were classified as complete responders when they showed a complete normalization of all baseline abnormal exams (clinical, biological and imaging). Non responders were defined by the absence of improvement of all baseline abnormal exams or an aggravation of at least one exam. All other cases were defined as partial responders.

Results: We analyzed 25 patients, aged 38 years, 36.4% had chest pain/heart failure, and abnormal findings on EKG (73%), cardiac MRI (55%), echocardiography (40%), 18 FDG PETscan (30%) and scintigraphy (10%). 42% had > 4 organs involved by sarcoidosis. Sarcoidosis duration before starting TNFI was 136 months [19; 311]. CS was refractory to other immunosuppressants i.e. methotrexate (n=24/25), cyclophosphamide (n=12/25), azathioprine (n=8/25), and mycophenolate mofetyl (n=6/25). After a follow up of 50.7 months after starting TNFI (infliximab n=24, etanercept n=1), 36% patients were complete responders, 48% partial responders and 16% non-responders. 8% had a CS relapse and were treated by a second course of TNFI with a good response. Corticosteroids were given at baseline in all patients; mean daily dose of steroids was 21 mg [5;50] at baseline versus 10 mg [1;40] at the last visit, and they were stopped in 28%. Eight patients had to withdraw TNFI because of adverse events, i.e. infection (n=5), allergy (n=1), and cardiac arrest (n=2). Two patients died, from a sudden death and an unknown cause (two months after stopping TNFI).

Conclusion: TNF alpha inhibitors showed a complete/partial cardiac response in 84% of patients with cardiac sarcoidosis refractory to immunosuppressive therapy, with a steroid sparing-effect. Adverse events led to TNF alpha inhibitors withdrawal in one third of patients.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/efficacy-and-tolerance-of-tnf-alpha-inhibitor-tnfi-treatment-in-cardiac-sarcoidosis-cs/