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Abstract Number: 2904

Efficacy and Tolerance of TNF Alpha Inhibitor (TNFI) Treatment in Cardiac Sarcoidosis (CS)

Deborah Puyraimond-Zemmour1, Catherine Chapelon-Abric2, David Saadoun3, Diane Bouvry4, Marc Ruivard5, Marc Andre6, Laurent Perard7, Pascal Sève8 and Patrice Cacoub9, 1Sorbonne Universités, UPMC Univ Paris 06, UMR 7211, and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), F-75005, Paris, France, Paris, France, 2AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, F-75013, Paris, France, Paris, France, 3Department of Internal Medicine and clinical Immunology. French National Reference Center for Autoimmune Diseases. DHU I2B (Inflammation, Immunotherapy and Biotherapy), UPMC, Paris VI, Hôpital Pitié Salpétrière, AP-HP, UPMC, Univ Paris 06, Paris, France, 4Hopital Avicenne, Department of Pneumology, Bobigny, France, Bobigny, France, 5CHU Estaing, Department of Internal Medicine, Clermont-Ferrand, France, Clermont Ferrand, France, 6CHU Montpied, Department of Internal Medicine, Clermont-Ferrand, France, Clermont Ferrand, France, 7Department of Internal Medicine, Centre Hospitalier Saint Joseph-Saint Luc, Lyon, France, 8Internal medicine, Internal medicine department, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, Lyon, France, 9Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Pitié-Salpêtrière, Paris, France

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Efficient, infliximab, safety and sarcoidosis

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Session Information

Date: Wednesday, November 8, 2017

Session Title: Miscellaneous Rheumatic and Inflammatory Diseases II

Session Type: ACR Concurrent Abstract Session

Session Time: 9:00AM-10:30AM

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.


Disclosure: D. Puyraimond-Zemmour, None; C. Chapelon-Abric, None; D. Saadoun, None; D. Bouvry, None; M. Ruivard, None; M. Andre, None; L. Perard, None; P. Sève, None; P. Cacoub, None.

To cite this abstract in AMA style:

Puyraimond-Zemmour D, Chapelon-Abric C, Saadoun D, Bouvry D, Ruivard M, Andre M, Perard L, Sève P, Cacoub P. Efficacy and Tolerance of TNF Alpha Inhibitor (TNFI) Treatment in Cardiac Sarcoidosis (CS) [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/efficacy-and-tolerance-of-tnf-alpha-inhibitor-tnfi-treatment-in-cardiac-sarcoidosis-cs/. Accessed July 5, 2022.
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