IgG4-Related Sclerosing Cholangitis in Europe

Published on Jan 1, 2019
· DOI :10.1007/978-981-10-4548-6_21
Nicolò de Pretis8
Estimated H-index: 8
(University of Verona),
Antonio Amodio13
Estimated H-index: 13
(University of Verona)
+ 1 AuthorsLuca Frulloni58
Estimated H-index: 58
(University of Verona)
Source
Abstract
IgG4-related sclerosing cholangitis (IgG4-RC) is a clinical condition described in the last 10 years, according to the growing knowledge on IgG4-related diseases (IgG4-RD). In Europe, few studies have been published on IgG4-RC and the information available are mainly derived from patients suffering from autoimmune pancreatitis (AIP). The chapter report the knowledge about the disease in Europe, and diagnostic algorytm and therapy currently applied for this rare but challenging disease.
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2019
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Objective Corticosteroid has been established as the standard therapy for autoimmune pancreatitis (AIP), but the requirement for maintenance corticosteroid therapy is controversial. We conducted a randomised controlled trial to clarify the efficacy of maintenance corticosteroid therapy in patients with AIP. Design We conducted a multicentre, tertiary setting, randomised controlled trial. After the induction of remission with the initial oral prednisolone (PSL) treatment, maintenance therapy with...
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Abstract Objective Autoimmune pancreatitis (AIP) responds dramatically to corticosteroids treatment. We reviewed our experience to determine the safety and effectiveness of treating obstructive jaundice in definitive AIP with corticosteroids alone without biliary stenting. Methods From our AIP database, we retrospectively identified type 1 AIP subjects whose jaundice was treated with corticosteroids alone without biliary stenting. Their medical records were reviewed and clinical data were evalua...
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#1Arezou Khosroshahi (Emory University)H-Index: 31
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Last. John H. Stone (Harvard University)H-Index: 94
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A. Khosroshahi, Z. S. Wallace, J. L. Crowe, T. Akamizu, A. Azumi, M. N. Carruthers, S. T. Chari, E. Della-Torre, L. Frulloni, H. Goto, P. A. Hart, T. Kamisawa, S. Kawa, M. Kawano, M. H. Kim, Y. Kodama, K. Kubota, M. M. Lerch, M. L€ ohr, Y. Masaki, S. Matsui, T. Mimori, S. Nakamura, T. Nakazawa, H. Ohara, K. Okazaki, J. H. Ryu, T. Saeki, N. Schleinitz, A. Shimatsu, T. Shimosegawa, H. Takahashi, M. Takahira, A. Tanaka, M. Topazian, H. Umehara, G. J. Webster, T. E. Witzig, M. Yamamoto, W. Zhang, T....
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Objectives To evaluate the efficacy of rituximab (RTX) in IgG4-related disease (IgG4-RD) in an open-label pilot trial. Methods We treated 30 IgG4-RD patients with two doses of RTX (1000 mg each). The participants were either treated with RTX alone (n = 26; 87%) or required to discontinue baseline glucocorticoids (GC) within 2 months (n = 4; 13%). Disease activity was measured by the IgG4-RD Responder Index (IgG4-RD RI) and physician9s global assessment (PGA). Disease response was defined as the ...
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#1Nicolò de Pretis (University of Verona)H-Index: 8
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OBJECTIVES:Steroids are used to induce remission in autoimmune pancreatitis (AIP). Low-dosage steroid therapy or immunosuppressant (IMs) has been proposed as maintenance therapy to prevent AIP relapse. Few and conflicting data have been published on the efficacy of azathioprine (AZA) in preventing A
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#1Jorie Buijs (Erasmus University Medical Center)H-Index: 6
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#1Marianne J. Van Heerde (Erasmus University Medical Center)H-Index: 7
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#1Tsukasa Ikeura (Kansai Medical University)H-Index: 19
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