Identification of relapse predictors in IgG4-related disease using multivariate analysis of clinical data at the first visit and initial treatment

Abstract

Objectives: Inducting clinical remission by glucocorticoid treatment is relatively easy in IgG4-related disease (IgG4-RD), but relapse also occurs easily with tapering of the steroid dose. The present study tried to analyse the cases to extract predictors of relapse present at the diagnosis of IgG4-RD.

Methods: Subjects comprised 79 patients with IgG4-related dacryoadenitis and sialadenitis, known as Mikulicz's disease, who were diagnosed between April 1997 and October 2013 and followed-up for >2 years from the initial induction treatment. They were applied to Cox proportional hazard modelling, based on the outcome of interval to relapse. We performed multivariate analysis for the clinical factors of these cases and identified predictors of relapse.

Results: Identified factors were male sex and younger onset in cases without organ involvement at diagnosis and low levels of serum IgG4 in cases with organ dysfunction at diagnosis. Complication with autoimmune pancreatitis and low steroid dose at initial treatment also tended to be associated with recurrence.

Conclusion: Follow-up is important in cases with recognized risk factors for relapse, including male sex and younger onset in cases without organ damage.

Keywords: IgG4-related disease; Mikulicz’s disease; autoimmune pancreatitis; multivariate analysis; relapse.