Open Label Two-Arm Study to Evaluate Rilzabrutinib (PRN1008) in IgG4-Related Disease Patients - NCT04520451

Approximately 25 patients with active IgG4-RD will be enrolled in two arms of the trial: (1) Experimental: PRN1008 with glucocorticoids and (2) Active Comparator: glucocorticoids only.

Patients who meet all the inclusion criteria and none of the exclusion criteria after completion of the 4-week Screening Period will be randomized in a 3:1 ratio to the PRN1008 with glucocorticoids arm or the glucocorticoids only arm. The Main Treatment Period is 12 weeks, and patients may then be eligible to enroll in a Treatment Extension Period of 40 weeks, followed by a 4-week Safety Follow-up Period.

In the Active Comparator arm, a patient will be considered a treatment failure if the glucocorticoid therapy cannot be discontinued by 12 weeks and will be eligible to cross-over to the Experimental arm, and receive 12 weeks of PRN1008 in the Main Treatment Period, followed by the potential to enroll in the Treatment Extension Period for an additional 40 weeks, followed by a 4-week Safety Follow-up Period.