Table 2.
Clinical trials and comparative effectiveness studies in IgG4-related disease
Study/trial | Study design a | Study arms | Primary outcome | Follow-up time | Results/status |
---|---|---|---|---|---|
Glucocorticoids | |||||
Masaki et al. (2017) [72] | Single-arm open-label trial | PSL 0.6 mg/kg/day tapered to maintenance ≤10 mg (median 7 mg/day), n = 61 | CR | 12 months |
|
Wu et al. (2017) [73] | Open-label RCT |
|
CR | 24 months |
|
Masamune et al. (2017) [74] | Open-label RCT (AIP) |
|
Relapse | 36 months | Relapse rate 61% (group 1) vs 24% (group 2), P = 0.007 |
Conventional synthetic DMARDs | |||||
Yunyun et al. (2017) [75] | Non-randomized clinical trial |
|
Relapse rate | 12 months |
|
Yunyun et al. (2019) [76] | Open-label RCT |
|
CR, PR | 12 months |
|
Wang et al. (2020) [77] | Open-label RCT |
|
Relapse | 12 months | Hazard ratio for time to relapse 0.35 (0.13, 0.90, P = 0.23), favouring group 2 |
Biologic DMARDs | |||||
Carruthers et al. (2015) [78] | Single-arm open-label trial | RTX 1 g × 2 doses, either monotherapy (n = 26) or with concomitant glucocorticoids tapered off over 2 months (n = 4) | At 6 months: decline in IgG4-RD RI, no relapses, and no GC use after 2 months | 12 months |
|
Ebbo et al. (2017) [79] | Retrospective cohort study |
|
Relapse | Mean 25 months |
|
Majumder et al. (2018) [80] | Retrospective cohort study (pancreaticobiliary IgG4-RD) |
|
Relapse rate | Median 34 months (group 1), 27 months (group 2) |
|
Campochiaro et al. (2020) [81] | Retrospective cohort study |
|
Relapse at 18 months | Median 26 months (group 1), 19 months (group 2a), 21 months (group 2b) | 71% relapse (group 1) vs 0% relapse (group 2), P = 0.006 |
Matza et al. (2022) [82] | Single-arm open-label trial | Abatacept 125 mg s.c. weekly, n = 10 | CR | 6 months |
|
Perugino et al. (2023) [83] | Single-arm, open-label trial | Obexelimab 5 mg/kg i.v. every 2 weeks with GCs discontinued within 2 months, n = 15 | Decline in IgG4-RD RI | 6 months |
|
Other | |||||
Zhang et al. (2019) [84] | Single-arm open-label trial (mild disease) | One i.m. injection of 5 mg betamethasone dipropionate with 2 mg betamethasone sodium phosphate + iguratimod 25 mg p.o. twice daily, n = 30 | CR, PR | 6 months |
|
Studies specific to AIP were limited to randomized controlled trials only.
AIP: autoimmune pancreatitis; CR: complete remission/response; GC: glucocorticoid; PDN: prednisone; PR: partial remission/response; PSL: prednisolone; RCT: randomized controlled trial; RI: responder index; RTX: rituximab; w/d: withdrawal.