Efficacy of Steroid Pulse Therapy for Autoimmune Pancreatitis Type 1: A Retrospective Study.

Mitsuru Sugimoto,Rei Suzuki,Yuichi Waragai,Jun Nakamura,Katsutoshi Obara,Hitomi Kikuchi,Hiroyuki Asama,Takuto Hikichi,Tadayuki Takagi,Naoki Konno,Ko Watanabe,Hiroshi Watanabe,Mika Takasumi,Hiromasa Ohira,Pavel Strnad

doi:10.1371/journal.pone.0138604

Mitsuru Sugimoto, Rei Suzuki + Show 13 more

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https://doi.org/10.1371/journal.pone.0138604

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Abstract

Autoimmune pancreatitis (AIP) is treatable with steroids, but relapse is frequent. The efficacy of steroid pulse therapy has been shown for various autoimmune diseases, but has not become established therapy. In this study, we reviewed the efficacy of steroid pulse therapy in 24 subjects who were diagnosed with AIP type 1 at our hospital. Patient characteristics, time-course of serum IgG4, and the cumulative relapse-free survival rate were compared between patients who received oral steroid therapy (oral group) and those who were treated with steroid pulse therapy (pulse group). Serum IgG4 was reduced significantly after therapy in both groups and the 5-year cumulative relapse-free survival rates in the two groups did not differ significantly (oral group 46.9%, pulse group 77.8%). However, in a subset of cases with diffuse pancreatic swelling, this rate was significantly lower in the oral group (33.3% vs. 100.0%, p = 0.046). These results suggest that steroid pulse therapy is effective for prevention of relapse in AIP patients with diffuse pancreatic swelling.

Highlights

Autoimmune pancreatitis (AIP) was defined by Yoshida et al as pancreatitis caused by irregular narrowing of the pancreatic duct, pancreatic swelling, or infiltration and fibrillation of lymphocytes, with such events related to autoimmune mechanisms [1]
In a subset of cases with diffuse pancreatic swelling, this rate was significantly lower in the oral group (33.3% vs. 100.0%, p = 0.046) (Fig 3B)
The long term prognoses of patients who received oral steroid therapy or steroid pulse therapy were not significantly different, but a protective effect against relapse was found after steroid pulse therapy in patients with diffuse pancreatic swelling

Summary

Introduction

Autoimmune pancreatitis (AIP) was defined by Yoshida et al as pancreatitis caused by irregular narrowing of the pancreatic duct, pancreatic swelling, or infiltration and fibrillation of lymphocytes, with such events related to autoimmune mechanisms [1]. The 2010 International Consensus Diagnostic Criteria (ICDC) for Autoimmune Pancreatitis [3] define pancreatitis as “Type 1” when other organ involvement and elevated serum IgG4 are present and lymphoplasmacytic sclerosing pancreatitis (LPSP) is histologically the distinguishing characteristic; or “Type 2” when elevated serum IgG4 is not present, the symptoms accompany inflammatory bowel disease, and idiopathic duct-centric chronic pancreatitis (IDCP) / granulocytic epithelial lesion (GEL) are histologically the distinguishing characteristics. Due to growing recognition of AIP, the number of reported cases has increased. Steroid therapy is the standard treatment, but relapse is reported to occur in 10–53% of cases [4,5,6,7].

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