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11.
Intern Med ; 56(22): 3023-3026, 2017 Nov 15.
Article in English | MEDLINE | ID: mdl-29021429

ABSTRACT

The patient was a 76-year-old woman who had noticed slight difficulty in swallowing in the 3 years prior to this presentation. Her dysphagia progressed while she was hospitalized following cervical cancer surgery. Esophagogastroduodenoscopy and an esophagram showed circumferential erosion and a stricture of the thoracic esophagus. Esophageal resection was performed; the resected specimens showed a stricture and wall thickening. Histologically, transmural hyperplasia, which consisted of inflammatory granulation tissue with the abundant infiltration of IgG4-positive plasma cells and lymphocytes, was observed. The patient was diagnosed with probable IgG4-related disease. IgG4-related esophageal disease presenting as esophageal lesions alone is a very rare condition.


Subject(s)
Autoimmune Diseases/pathology , Esophagitis/pathology , Immunoglobulin G/blood , Plasma Cells/immunology , Aged , Autoimmune Diseases/blood , Esophagitis/blood , Female , Humans , Plasma Cells/pathology
18.
Pancreatology ; 14(5): 373-9, 2014.
Article in English | MEDLINE | ID: mdl-25278307

ABSTRACT

OBJECTIVES: Ordinary chronic pancreatitis (CP), such as alcoholic CP, is well established to have the increased risk for pancreatic cancer (PaC), nevertheless an association between autoimmune pancreatitis (AIP) and PaC is still unknown. The aims of this study are to examine the frequency of patients who developed PaC during follow-up after being diagnosed with type 1 AIP and to compare the incidence rate of PaC between patients with type 1 AIP and CP. METHODS: Sixty-three patients with type 1 AIP and 41 patients with CP were enrolled. We examined development of PaC during follow-up from their clinical records. RESULTS: The mean follow-up period was 62.4 months in AIP group and 49.2 months in CP group. The occurrence of PaC was observed in 3 patients with AIP during the mean follow-up period of 94.7 months (range, 31-186), whereas a single CP patient developed PaC 38 months after CP diagnosis. The incident rate of PaC during follow-up was comparable between the 2 groups [4.8% (3/63) in type 1 AIP group vs. 2.4% (1/41) in CP group]. In all of 3 AIP patients who developed accompanying PaC, the clinical remission of AIP was achieved with maintenance steroid therapy, when tumors were discovered. In the histological examination of one surgical patient with PaC, lymphoplasmacytic infiltration in storiform fibrosis with abundant IgG4-positive cell infiltration was observed around the PaC area. CONCLUSIONS: Similar to patients with ordinary CP, surveillance for development of PaC is needed at regular interval during follow-up in AIP patients.


Subject(s)
Adenocarcinoma/etiology , Autoimmune Diseases/complications , Pancreatic Neoplasms/etiology , Pancreatitis/complications , Adenocarcinoma/diagnosis , Adenocarcinoma/epidemiology , Adult , Aged , Aged, 80 and over , Case-Control Studies , Female , Follow-Up Studies , Humans , Incidence , Male , Middle Aged , Pancreatic Neoplasms/diagnosis , Pancreatic Neoplasms/epidemiology , Pancreatitis/immunology , Pancreatitis, Chronic/complications , Risk Factors
19.
Pancreatology ; 14(3): 193-200, 2014.
Article in English | MEDLINE | ID: mdl-24854615

ABSTRACT

BACKGROUND: Patients with type 1 autoimmune pancreatitis (AIP) have several immunologic and histologic abnormalities. It is known that depletion of B cells by rituximab is effective for treatment of IgG4-related disease (IgG4-RD) such as type 1 AIP, suggesting that B cells may be a key player in IgG4-RD. However, the role of regulatory B cells (Bregs) in type 1 AIP is unclear, and the objective of this paper is to clarify the role of Bregs in the pathophysiology of type 1 AIP by analyzing circulating Bregs. METHOD: We recruited 21 patients with type 1 AIP as determined by the International Consensus Diagnostic Criteria for AIP (ICDC). No patients received corticosteroid treatments. For comparison, we recruited 14 patients with chronic pancreatitis (CP), 20 patients with pancreatic cancer, and 25 healthy subjects as controls. We analyzed Bregs as CD19+ CD24high CD38high and CD19+ CD24high CD27+ from peripheral blood by flow cytometry. RESULTS: In peripheral blood, CD19+ CD24high CD38high Bregs were significantly increased in type 1 AIP patients compared with CP, pancreatic cancer, and healthy controls. Although not significant different, CD19+ CD24high CD27+ Bregs of type 1 AIP were decreased compared to those of other groups. IL-10(+) B cells were not significantly different from type 1 AIP patients and healthy controls. In untreated type 1 AIP patients, the number of CD19+ CD24high CD38high Bregs and IgG4 were not correlated. CONCLUSIONS: Our data suggested that CD19+ CD24high CD38high Bregs seemed to increase reactively to suppress the disease activity, and are consistent with the hypothesis that CD19+ CD24high CD27+ Bregs might be involved in the development of type 1 AIP, although it still remains unclear whether the decrease of CD19+ CD24high CD27+ cells is cause or effect of AIP.


Subject(s)
Autoimmune Diseases/immunology , B-Lymphocytes, Regulatory/metabolism , Pancreatitis/immunology , ADP-ribosyl Cyclase 1/blood , Adult , Aged , Aged, 80 and over , Antigens, CD19/blood , Biomarkers/blood , CD24 Antigen/blood , Case-Control Studies , Female , Flow Cytometry , Humans , Interleukin-10/blood , Male , Membrane Glycoproteins/blood , Middle Aged , Tumor Necrosis Factor Receptor Superfamily, Member 7/blood
20.
J Gastroenterol ; 49(1): 156-62, 2014 Jan.
Article in English | MEDLINE | ID: mdl-23955155

ABSTRACT

BACKGROUND: Clinical evidence regarding intestinal Behçet's disease (BD) management is lacking and intestinal lesions are a poor prognostic factor. In 2007, the Japan consensus statement for diagnosis and management of intestinal BD was developed. Recently, the efficacy of anti-tumor necrosis factor (TNF)α monoclonal antibodies (mAbs), and infliximab (IFX) was reported and adalimumab (ADA) was approved for intestinal BD in Japan. This study renewed consensus-based practice guidelines for diagnosis and treatment of intestinal BD focusing on the indication of anti-TNFα mAbs. METHODS: An expert panel of Japanese gastroenterology and rheumatology specialists was involved. Clinical statements for ratings were extracted from the literature, a professional group survey, and by an expert panel discussion, which rated clinical statements on a nine-point scale. After the first round of ratings, a panelist meeting discussed areas of disagreement and clarified areas of uncertainty. The list of clinical statements was revised after the panelist meeting and a second round of ratings was conducted. RESULTS: Fifteen relevant articles were selected. Based on the first edition consensus statement, improved clinical statements regarding indications for anti-TNFα mAbs use were developed. After a two-round modified Delphi approach, the second edition of consensus statements was finalized. CONCLUSIONS: In addition to standard therapies in the first edition, anti-TNFα mAbs (ADA and IFX) should be considered as a standard therapy for intestinal BD. Colchicines, thalidomide, other pharmacological therapy, endoscopic therapy, and leukocytapheresis were deemed experimental therapies.


Subject(s)
Antibodies, Monoclonal/therapeutic use , Behcet Syndrome/diagnosis , Gastrointestinal Agents/therapeutic use , Intestinal Diseases/diagnosis , Tumor Necrosis Factor-alpha/antagonists & inhibitors , Behcet Syndrome/drug therapy , Delphi Technique , Humans , Intestinal Diseases/drug therapy , Practice Guidelines as Topic
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