Assessing disease relapse post cessation of immunosuppressive therapy in patients with cardiac sarcoidosis

SESSION TITLE: Diffuse Lung Disease: Reimagining the Standard of Care SESSION TYPE: Rapid Fire Original Inv PRESENTED ON: 10/10/2023 12:00 pm - 12:45 pm PURPOSE: Sarcoidosis is a systemic granulomatous disease, which significantly affects the heart in less than 5% of patients. The optimal duration of immunosuppressive therapy in cardiac sarcoidosis is unknown, with a systematic review and meta-analysis of 10 cohort studies showing that the duration of corticosteroid treatment ranged from 3 to 168 months. In that meta-analysis, the association between duration of immunosuppressive therapy and disease recurrence was not evaluated. We aim to assess the incindence of disease relapse post cessation of immunosuppression and determine if the duration of immunosuppression varied significantly in those with disease relapse compared to disease remission. METHODS: We prospectively included patients clinically judged to have cardiac sarcoidosis, by a multidisciplinary sarcoidosis team, from November 2016 to June 2021. We included only incident cases. We retrospectively collected longitudinal data until January of 2023. Relapse was defined as a cardiac PET scan with focal FDG uptake after cessation of immunosuppression, and a previously negative cardiac PET. If no cardiac PET was available after cessation of immunosuppression, clinical data was used to evaluate relapse, such as worsening of heart block, arrythmias or heart failure. RESULTS: Amongst the 212 patients in the registry, we included 36 patients (17%) who had stopped immunosuppression. Patients were predominantly male (23/36, 64%) and white (29/36, 81%). Median age was 55 years (range: 30 to 77 years). Clinical presentations included AV block (16/36, 44%), heart failure (12/36, 33%), and/or ventricular arrythmias (4/36, 11%). Most (26/36, 72%) had FDG uptake on baseline cardiac PET, and 47% (17/36) had delayed enhancement on baseline cardiac MRI. Initial therapies included prednisone (32/36, 90%), methotrexate (22/36, 61%), and/or leflunomide (3/36, 8%). Median duration of immunosuppression was 25 months (range: 3 to 66 months). Amongst 15 patients with a cardiac PET available post cessation of immunosuppression, only 2 (13%) had evidence of relapse. Duration of immunosuppression was not significantly different between relapse and remission groups (31.5 versus 25.5 months, respectively, p = 1.000). Both patients with relapse were initially treated with combination therapy, comprising of prednisone and methotrexate. One had initially presented with complete heart block and the other with heart failure. Amongst 21 patients with no cardiac PET scan data post cessation of immunosuppression, 15 patients had no evidence of clinical relapse, and 6 were lost to follow up. CONCLUSIONS: The rate of relapse was low in our cohort. We were able to safely stop immunosuppression in some patients, however the duration of immunosuppression varied widely in the cohort. We found no statistically significant association between relapse and duration of initial regimen of immunosuppression, although the small sample size limits our conclusions. CLINICAL IMPLICATIONS: This retrospective study signals that immunosuppression can be safely stopped in select patients with cardiac sarcoidosis. This contrasts with a previous retrospective study, which had a significantly higher rate of relapse (88%) after cessation of immunosuppression, post 24 months of therapy. Larger sample sizes are needed to draw more definitive conclusions regarding ideal duration of immunosuppression. DISCLOSURES: No disclosure on file for Paul Cremer No disclosure on file for Daniel Culver No relevant relationships by Simran Ganeriwal No relevant relationships by Christine Jellis No relevant relationships by Manuel Ribeiro Neto No relevant relationships by Tarek Souaid No relevant relationships by Ziad Taimeh