Table 1.
Design and baseline demographics of the included studies.
| Author, year | Country | Study type | Publication type | Years | Study population | Non-steroidal treatment studied (n) | Patients receiving non-steroidal treatment (n) | Age (years) | Female sex (n, %) | Race (n, %) |
|---|---|---|---|---|---|---|---|---|---|---|
| Synthetic disease-modifying antirheumatic drugs (sDMARD) | ||||||||||
| Yazaki et al, 2014 [19] | Japan | Retrospective, single-center cohort | abstract | N/A | CS patients with addition of MTX due to relapse, deterioration or steroid-related adverse effects | MTX | 7 | N/A | N/A | N/A |
| Yokomatsu et al, 2018 [20] | Japan | Retrospective, single-center cohort | abstract | N/A | CS patients treated with MTX with sequential PET scans at least 12 months apart | MTX | 6 | mean: 66 | 4 (66.7%) | N/A |
| Nagai et al, 2014 [21] | Japan | Retrospective, single-center cohort | full | N/A | CS patients followed every three months for five years in the CS clinic | MTX | 10 | 65.9 ± 7.7 | 8 (80%) | N/A |
| Ballul et al, 2019 [23] | France | Retrospective, single-center cohort | full | 2012–2016 | Consecutive patients with histologically proven sarcoidosis | MTX (5, 41.7%), AZA (5, 41.7%), CP (n = 2, 16.7%) | 12 | 50.6 (mean) | 6 (50%) | Black (12, 100%) |
| Chapelon-Abric et al, 2017 [24] | France | Retrospective, single-center cohort | full | 1995–2014 | Patients with CS treated in a single department | CP (20, 57.1%), MTX (12, 34.3%), MMF (2, 5.7%), cyclosporine A (1, 2.9% - transplant) | 35 | median: 42 (95% CI: 33–49) | 9 (25.7%) | Caucasian: 22 (63%), Black: 12 (34%), Asian: 1(3%) |
| Fussner et al, 2016 [25] | USA | Retrospective, two-center cohort | abstract | 1994–2014 | Patients with CS who received MMF in two large academic centers | MMF | 33 | median: 51 [IQR: 47–58] | 9 (27%) | Caucasian (26, 79%) |
| Griffin et al, 2018 [26] | USA | Retrospective, single-center cohort | abstract | N/A | CS patients who received combination therapy of MMF with prednisone | MMF | 25 | 51.5 ± 11.4 | 10 (40%) | Black: 10 (40%) |
| Biological disease-modifying antirheumatic drugs (bDMARD) | ||||||||||
| Rosenthal et al, 2019 [22] | USA | Retro-/prospective, single-center cohort | full | 2009–2018 | Treatment-naïve CS patients with two consecutive cardiac PET scans (6 months apart) | MTX (25) ± ADA (19, if persistent symptoms or intolerance to MTX) | 28 | 52 | 12 (42.8%) | N/A |
| Sethi et al, 2018 [38] | USA | Retrospective, single-center cohort | abstract | N/A | CS patients with at least two sequential cardiac PET scans | MTX (15, 100%), ADA (added in 8 [53%]) | 15 | N/A | N/A | N/A |
| Estephan et al, 2017 [27] | USA | Retrospective, single-center study | abstract | 2013–2014 | Sarcoidosis patients with cardiac PET suggesting cardiac involvement | IFX (8, 53.3%), ADA (1, 6.7%), MMF (7, 46.7%), AZA (2, 13.3%) | 15 | N/A | N/A | N/A |
| Kandolin et al, 2017 [28] | Finland | Retrospective, single-center cohort | abstract | 2012–2017 | Biopsy-proven CS patients receiving IFX as fourth-line treatment due to persistent disease activity, adverse events or intolerance to other medications | IFX | 9 | 53 ± 11.1 | 6 (67%) | N/A |
| Kowlgi et al, 2019 [29] | International | Retrospective, multi-center cohort | abstract | N/A | Refractory CS that have failed treatment with at least one immunosuppressant | IFX | 27 | 54 [45–59] | 8 (29.6%) | N/A |
| Chapelon-Abric et al, 2015 [30] | France | Retrospective, single-center cohort | full | 2005–2013 | Consecutive patients with biopsy-proven, severe and treatment-resistant sarcoidosis with cardiac and/or neuro involvement | IFX | 16 | median: 36 [range: 26–43] | 7 (43.8%) | Caucasian: 10 (62.5%), Black: 5 (31.3%), Asian: 1 (6.3%) |
| Harper et al, 2019 [31] | USA | Retrospective, single-center cohort | full | N/A | CS patients on IFX due to refractory arrythmias or persistently elevated 18F-FDG uptake with cardiac symptoms | IFX | 36 | 50 ± 11 | 10 (27.8%) | White (28, 77.8%); Black (8, 22.2%) |
| Cundiff et al, 2019 [32] | USA | Retrospective, single-center cohort | abstract | N/A | Patients with metabolically active CS (defined by cardiac PET) treated with TNFi due to disease progression or intolerance, contraindications to steroids. | IFX (8, 88.9%), ADA (1, 11.1%) | 9 | N/A | N/A | N/A |
| Sinokrot et al, 2019 [33] | USA | Retrospective, single-center cohort | abstract | 2016–2018 | Refractory CS disease (dysrhythmias, cardiomyopathy and persistent 18F-FDG uptake) despite 1st/2nd-line therapies | IFX | 5 | N/A | N/A | N/A |
| Devraj et al, 2020 [34] | USA | Retro-/prospective, single-center cohort | abstract | 2013–2018 | All CS patients treated with biologics due to disease progression or intolerance, contraindications to standard therapy.All had received steroids prior to initiation | ADA (7, 58.3%), IFX (4, 33.3%) and RXM (1, 8.3%) | 12 | 52 ± 8 | 50% | Black (9, 74%); White (3, 25%) |
| Puyraimond-Zemmour et al, 2017 [35] | France | Retrospective, multi-center cohort | abstract | N/A | Patients with definite histologically proven extra-thoracic sarcoidosis involving the heart who received a TNFi | IFX (24, 96%), ETN (1, 4%) | 25 | 38 | N/A | N/A |
| Jamilloux et al, 2017 [15] | France | Retrospective, multi-center cohort | full | 2014–2015 | Sarcoidosis patients treated with anti-TNF agents (28/132 [21.2%] had cardiac involvement) | IFX (120, 91%), ADA (8, 6%), ETN (3, 2%), CZP (1, 1%) | 132 (28 with CS) | mean: 45.5 [range: 14–78] | 76 (57.6%) | Caucasian: 88 (66.7%), Black: 37 (28%), Asian: 4 (3%), N/A: 3 (2.2%) |
| Krause et al, 2016 [41] | USA | Retrospective, single-center cohort | abstract | N/A | All CS cases treated with RXM due to failure of 1st/2nd-line treatment (corticosteroids ± MMF (80%), MTX (40%), AZA (20%), IFX (20%), leflunomide (20%)) with ≥ 1 follow-up | RXM | 5 | 50.9 ± 8.8 | 2 (40%) | N/A |
| Baker et al, 2019 [37] | USA | Retrospective, single-center cohort | full | 2009–2018 | All CS cases treated with TNFi for worsening imaging findings | IFX (10, 50%), ADA (10,50%-one patient had received IFX) Golimumab (1, 5%) | 77 (TNFi only 20) | Mean 55 (median 58 years) | 39% | 66% Whitie, 16% Black, 9% Asians, 9% Hispanics |
| Gilotra et al, 2020 [39] | USA | Retrospective, multi-center | full | 2014–2019 | All CS were treated with TNFi for 1) persistent cardiac inflammation on FDG-PET despite immunosuppression 2) clinically active CS and/or 3) into side effects from immunosuppression agents. | IFX (30, 79%), ADA (8, 21%) | 38 | Mean 49.9 | 42% | 53% Black |
| Injean et al, 2019 [36] | USA | Retrospective, single center | abstract | 2014–2019 | Not specified. | Multiple. IFX (3, 21%), ADA (2, 14%). Also, steroids, AZA, MTX, MMF, HCQ, CP, tacrolimus | 14 | 58 | 40% | N/A |
ADA: adalimumab; AZA: azathioprine; CP: cyclophosphamide; CS: cardiac sarcoidosis; CZP: certolizumab pegol; ETN: etanercept; FDG: fluorodeoxyglucose; HCQ: hydroxychloroquine; IFX: infliximab; MMF: mycophenolate mofetil; MTX: methotrexate; N/A: not available; PET: positron emission tomography; RXM: rituximab; TNF(i): tumor necrosis factor (inhibitor). Data presented as mean ± standard deviation and n (%) unless specified otherwise.