Abstract
BackgroundThe aim of this study was to assess the outcomes of remission induction in patients with IgG4-related disease (IgG4-RD) in our cohort, and to investigate the characteristics, prognosis, and risk factors in the patients failed of remission induction.MethodsWe prospectively enrolled 215 newly diagnosed patients with IgG4-RD, who were initially treated with glucocorticoid (GC) alone or in combination with immunosuppressive agents (IM), and had at least 6 months of follow up. The therapeutic goals of remission induction were defined as fulfilling each of the following after the 6-month remission induction stage: (1) ≥ 50% decline in the IgG4-RD responder index (RI); (2) GC tapered to maintenance dose; and (3) no relapse during GC tapering. The patients not achieving the therapeutic goals were considered to have failed of remission induction.ResultsThere were 26 patients in our cohort who failed of remission induction, including 16 (20.8%) on GC monotherapy, and 10 (7.2%) on combination therapy comprising GC and IM. The lacrimal gland and lung were most common sites of remission induction failure. Among the patients who relapsed during remission induction stage, 52.9% had secondary relapse during follow-up. Eosinophilia, higher baseline RI, more than five organs involved and dacryoadenitis were risk factors for remission induction failure with GC monotherapy, and the incidence of remission induction failure was 71.4% in the patients with more than three risk factors. After 6-month treatment, the patients who failed of remission induction had significantly higher erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and IgG4.ConclusionIn our cohort, 20.8% of patients failed of remission induction with GC monotherapy, while 7.2% of patients failed of remission induction with combination therapy comprising GC and IM.
Highlights
The aim of this study was to assess the outcomes of remission induction in patients with IgG4-related disease (IgG4-RD) in our cohort, and to investigate the characteristics, prognosis, and risk factors in the patients failed of remission induction
The most common clinical manifestations included Mikulicz’s disease, autoimmune pancreatitis, Patients who failed of remission induction As shown in Table 2, according to our definition, there were 26 (12.1%) patients in total who failed of remission induction, including 3 patients with decline in the IgG4-RD responder index (RI)
IgG4-RD IgG4-related disease, RI responder index, ESR erythrocyte sedimentation rate, CRP hyper-sensitivity C-reactive protein, GC glucocorticoid, IM immunosuppressive agents aHere we considered the maximum reduction during follow up, so that re-elevation due to relapse was not taken into account
Summary
The aim of this study was to assess the outcomes of remission induction in patients with IgG4-related disease (IgG4-RD) in our cohort, and to investigate the characteristics, prognosis, and risk factors in the patients failed of remission induction. Glucocorticoid (GC) is the standard first-line agent for remission induction [5]. Patients with IgG4-RD typically respond well to initial GC treatment, with improvement of symptoms and signs, resolution of enlarged organs and decrease in serum IgG4 levels [6,7,8,9,10]. There is a small minority of patients with less favorable response to remission induction, including those who have persistently active disease despite treatment, relapse during GC tapering or fail of GC tapering because of unstable disease [12, 17,18,19,20]. There are limited data on patients with less favorable response
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