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      Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis

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          Abstract

          Retroperitoneal fibrosis (RPF) is an uncommon condition characterized by inflammation and fibrosis in the retroperitoneal space. More than two-thirds of RPF are idiopathic, with the remaining stemed from a variety of secondary causes. It was suggested that IgG4-related RPF is a secondary form of RPF. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-related RPF and IRPF in a large Chinese cohort. We retrospectively reviewed the medical records of 132 RPF patients diagnosed at Peking University People’s Hospital between March 2010 and March 2018. Among the 132 patients, the mean age at disease onset was 54.8 years. IgG4-related RPF group showed greater male predominance compared to IRPF group. IgG4-related RPF patients showed a longer interval between symptom onset and diagnosis, and allergic diseases were more common in this group. Sixty-four patients (48.4%) had lower back pain, which was more common in IRPF group than that in IgG4-related RPF patients. In terms of organ involvement, although 42 of 47 patients (89.3%) with IgG4-related RPF had other organ involvement, there were no patients in the IRPF group with other organ involvement. In addition, the serum IgG4 level, elevated eosinophils counts and IgE level were significantly higher in IgG4-related RPF patients. We described the demographic, clinical and laboratory differences between IgG4-related RPF and IRPF patients, indicating their potential differences in pathogenesis, which was of great importance to diagnose and manage the two phenotypes.

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          IgG4-related disease.

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            IgG4-Related Disease: Clinical and Laboratory Features in One Hundred Twenty-Five Patients.

            IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect nearly any organ. Prior studies have focused on individual cases of IgG4-RD or small case series. This study was undertaken to report detailed clinical and laboratory findings in a larger group of patients with IgG4-RD whose diagnosis was established by strict clinicopathologic correlation.
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              Standard steroid treatment for autoimmune pancreatitis.

              To establish an appropriate steroid treatment regimen for autoimmune pancreatitis (AIP). A retrospective survey of AIP treatment was conducted in 17 centres in Japan. The main outcome measures were rate of remission and relapse. Of 563 patients with AIP, 459 (82%) received steroid treatment. The remission rate of steroid-treated AIP was 98%, which was significantly higher than that of patients without steroid treatment (74%, 77/104; p<0.001). Steroid treatment was given for obstructive jaundice (60%), abdominal pain (11%), associated extrapancreatic lesions except the biliary duct (11%), and diffuse enlargement of the pancreas (10%). There was no relationship between the period necessary to achieve remission and the initial dose (30 mg/day vs 40 mg/day) of prednisolone. Maintenance steroid treatment was given in 377 (82%) of 459 steroid-treated patients, and steroid treatment was stopped in 104 patients. The relapse rate of patients with AIP on maintenance treatment was 23% (63/273), which was significantly lower than that of patients who stopped maintenance treatment (34%, 35/104; p = 0.048). From the start of steroid treatment, 56% (55/99) relapsed within 1 year and 92% (91/99) relapsed within 3 years. Of the 89 relapsed patients, 83 (93%) received steroid re-treatment, and steroid re-treatment was effective in 97% of them. The major indication for steroid treatment in AIP is the presence of symptoms. An initial prednisolone dose of 0.6 mg/kg/day, is recommend, which is then reduced to a maintenance dose over a period of 3-6 months. Maintenance treatment with low-dose steroid reduces but dose not eliminate relapses.
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                Author and article information

                Contributors
                Role: ConceptualizationRole: Data curationRole: Formal analysisRole: MethodologyRole: Writing – original draft
                Role: ConceptualizationRole: Data curationRole: Formal analysisRole: MethodologyRole: Writing – review & editing
                Role: Data curationRole: MethodologyRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: MethodologyRole: Software
                Role: Resources
                Role: Data curation
                Role: Supervision
                Role: Software
                Role: Formal analysis
                Role: ConceptualizationRole: Formal analysisRole: Funding acquisitionRole: InvestigationRole: MethodologyRole: SupervisionRole: Writing – review & editing
                Role: Editor
                Journal
                PLoS One
                PLoS One
                plos
                plosone
                PLoS ONE
                Public Library of Science (San Francisco, CA USA )
                1932-6203
                18 February 2021
                2021
                : 16
                : 2
                : e0245601
                Affiliations
                [1 ] Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing, China
                [2 ] Department of Rheumatology and Immunology, Tengzhou Central People’s Hospital, Tengzhou, China
                [3 ] Department of Rheumatology and Immunology, Zhengzhou Central Hospital Affiliated to Zhengzhou University, Zhengzhou, China
                [4 ] Department of Geriatrics, Affiliated Hospital of North China University of Technology, Tangshan, China
                [5 ] Department of Ultrasound, Peking University People’s Hospital, Beijing, China
                [6 ] Department of Pathology, Peking University People’s Hospital, Beijing, China
                [7 ] Department of Radiology, Peking University People’s Hospital, Beijing, China
                Leiden University Medical Center, NETHERLANDS
                Author notes

                Competing Interests: The authors have declared that no competing interests exist.

                Author information
                https://orcid.org/0000-0003-2609-4052
                Article
                PONE-D-20-18066
                10.1371/journal.pone.0245601
                7891782
                33600452
                6e29ecc8-9898-4360-8741-2a7475293ab7
                © 2021 Wang et al

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 2 July 2020
                : 4 January 2021
                Page count
                Figures: 1, Tables: 5, Pages: 10
                Funding
                Funded by: Peking University People’s Hospital Research and Development Funds
                Award ID: RDX 2019-02
                Award Recipient :
                Funded by: Peking University People’s Hospital Research and Development Funds
                Award ID: (RDH2020-03
                Award Recipient :
                YY-L receive the fund: Peking University People’s Hospital Research and Development Funds (RDX 2019-02), and Peking University People’s Hospital Research and Development Funds (RDH2020-03), The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
                Categories
                Research Article
                Medicine and Health Sciences
                Pharmaceutics
                Drug Therapy
                Steroid Therapy
                Glucocorticoid Therapy
                Medicine and Health Sciences
                Surgical and Invasive Medical Procedures
                Biology and Life Sciences
                Developmental Biology
                Fibrosis
                Medicine and Health Sciences
                Clinical Medicine
                Clinical Immunology
                Allergies
                Allergic Diseases
                Biology and Life Sciences
                Immunology
                Clinical Immunology
                Allergies
                Allergic Diseases
                Medicine and Health Sciences
                Immunology
                Clinical Immunology
                Allergies
                Allergic Diseases
                Medicine and Health Sciences
                Surgical and Invasive Medical Procedures
                Biopsy
                Medicine and Health Sciences
                Diagnostic Medicine
                Clinical Laboratory Sciences
                Clinical Laboratories
                Medicine and Health Sciences
                Clinical Medicine
                Signs and Symptoms
                Pain
                Lower Back Pain
                Medicine and Health Sciences
                Clinical Medicine
                Signs and Symptoms
                Pain
                Abdominal Pain
                Custom metadata
                ll relevant data are within the manuscript and its Supporting information files.

                Uncategorized
                Uncategorized

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