Table 3.

Ongoing clinical trials in IgG4-related disease

Trial	Design	Study arms	Primary outcome	Follow-up time	Results/status
Conventional synthetic DMARDs
NCT05746689	Open-label, single-arm trial	Sirolimus + PDN taper	Relapse rate	3 months	Pre-enrolment
Biologic DMARDs
NCT04918147	Part 1: open-label, single-arm trial Part 2: placebo-controlled RCT	Part 1a/1b: elotuzumab (various regimens) + PDN taper Part 2: elotuzumab + PDN taper vs placebo + PDN taper	Part 1: adverse events Part 2: change in IgG4-RD RI	Part 1: up to 48 weeks Part 2: 48 weeks	Part 1 b enrolling
NCT05662241	Placebo-controlled RCT	Obexelimab + PDN taper vs placebo + PDN taper	Time to relapse	12 months	Enrolling
NCT04660565	RCT	Belimumab + GC vs GC monotherapy	Relapse rate	12 months	Enrolling
NCT05728684	Open-label single-arm trial	CM310 (anti-IL-4 receptor-α mAb)	Response rate	3 months	Pre-enrolment
NCT04540497	Placebo-controlled RCT	i.v. inebilizumab or placebo followed by optional 3-year open-label treatment period	Time to relapse	12 months	Active, no longer enrolling
NCT02705638	Open-label single-arm trial	Rituximab + lenalidomide	Remission	24 months	Completed
Targeted synthetic DMARDs
NCT05625581	Non-randomized controlled trial	Tofacitinib + GC taper vs CYC + GC taper	Remission	6 months	Enrolling
NCT04602598	Open-label single-arm trial	Zanubrutinib	Submandibular and lacrimal gland volume	6 months	Enrolling
NCT04520451	Open-label two-arm trial	Group 1: rilzabrutinib + 12 week GC taper Group 2: placebo + 12 week GC taper followed by crossover to rilzabrutinib	Relapse	12 months	Enrolling
NCT05781516	RCT	Baricitinib + GC taper vs GC taper monotherapy	Relapse	12 months	Enrolling

GC: glucocorticoid; IgG4-RD RI: IgG4-related disease responder index; PDN: prednisone; RCT: randomized controlled trial.