Table 2

Definitions used in this study for treatment response and resistance

Treatment response
Complete remissionFulfilment of each of the following: (1) resolution of clinical, radiographic and biochemical (ie, abnormal liver tests) evidence of inflammation in the pancreas and other involved organs; (2) absence of new inflammatory lesions at follow-up; and (3) discontinuation of therapies for disease control (such as steroids and biliary stents). Normalisation of serum IgG4 levels was not a requirement for a patient's disease to be considered in remission
Partial remissionImprovement without resolution of inflammatory changes (clinical, radiographic or biochemical), without an ongoing need for steroid therapy. This precedes complete remission, however its persistence beyond 4 months of RTX therapy requires further investigation
Incomplete remissionImprovement of inflammatory changes (clinical, radiographic or biochemical), with an ongoing need for RTX plus concurrent therapy (steroids or biliary stents). This is often seen in the first 6 weeks after starting RTX
RecrudescenceA flare of disease which is not yet in remission, typically occurring during a steroid taper
Treatment resistance
Steroid dependenceNeed for treatment with moderate doses of steroids (≥15 mg prednisone daily) for a prolonged period of time (>3 months) to maintain disease control
Steroid intoleranceInability to tolerate steroids due to either treatment-limiting side effects or exacerbation of premorbid co-morbidities (such as severe osteoporosis, unstable blood glucose levels, previous steroid-induced psychosis)
Immunosuppressant resistanceInability to discontinue steroids despite at least 3 months of immunosuppressive treatment, or occurrence of relapse while on immunosuppressive monotherapy