Type 1 Autoimmune Pancreatitis in Europe: Clinical Profile and Response to Treatment

被引:14
作者
Overbeek, Kasper A. [1 ]
Poulsen, Jakob L. [2 ]
Lanzillotta, Marco [3 ]
Vinge-Holmquist, Olof [4 ,5 ]
Macinga, Peter [6 ]
Demirci, A. Fatih [7 ]
Sindhunata, Daniko P. [8 ]
Backhus, Johanna [9 ]
Alguel, Hana [10 ]
Buijs, Jorie [1 ]
Levy, Philippe [11 ]
Kiriukova, Mariia [12 ]
Goni, Elisabetta [13 ]
Hollenbach, Marcus [14 ]
Miksch, Rainer C. [15 ]
Kunovsky, Lumir [16 ,17 ,18 ]
Vujasinovic, Miroslav [19 ]
Nikolic, Sara [19 ]
Dickerson, Luke [20 ]
Hirth, Michael [21 ]
Neurath, Markus F. [22 ]
Zumblick, Malte [23 ]
Vila, Josephine [24 ]
Jalal, Mustafa [25 ]
Beyer, Georg [12 ]
Frost, Fabian [26 ]
Carrara, Silvia [27 ]
Kala, Zdenek [17 ]
Jabandziev, Petr [28 ,29 ]
Sisman, Gurhan [30 ]
Akyuz, Filiz [31 ]
Capurso, Gabriele [32 ]
Falconi, Massimo [33 ]
Arlt, Alexander [9 ,34 ,35 ]
Vleggaar, Frank P. [36 ]
Barresi, Luca [37 ]
Greenhalf, Bill [19 ]
Czako, Laszlo [38 ]
Hegyi, Peter [38 ,39 ]
Hopper, Andrew [24 ]
Nayar, Manu K. [23 ]
Gress, Thomas M. [22 ]
Vitali, Francesco [21 ]
Schneider, Alexander [20 ]
Halloran, Chris M. [19 ]
Trna, Jan [40 ]
Okhlobystin, Alexey, V [41 ]
Dagna, Lorenzo [3 ]
Cahen, Djuna L. [1 ]
Bordin, Dmitry [42 ]
机构
[1] Erasmus MC, Univ Med Ctr, Dept Gastroenterol & Hepatol, Rotterdam, Netherlands
[2] Aalborg Univ Hosp, Ctr Pancreat Dis, Dept Gastroenterol & Hepatol, Aalborg, Denmark
[3] Ist Sci San Raffaele, Unit Immunol Rheumatol Allergy & Rare Dis UnIRAR, Milan, Italy
[4] Akershus Univ Hosp, Dept Digest Surg, Loerenskog, Norway
[5] Trondheim Reg & Univ Hosp, St Olavs Hosp, Dept Digest Surg, Trondheim, Norway
[6] Inst Clin & Expt Med, Dept Gastroenterol & Hepatol, Prague, Czech Republic
[7] Marmara Univ, Res & Educ Hosp, Dept Internal Med, Istanbul, Turkiye
[8] Univ Amsterdam, Amsterdam UMC, Dept Gastroenterol & Hepatol, Amsterdam, Netherlands
[9] Univ Hosp Ulm, Dept Internal Med 1, Ulm, Germany
[10] Tech Univ Munich, Dept Med 2, Munich, Germany
[11] Beaujon Hosp, APHP, Pancreatol Unit, Clichy, France
[12] AS Loginov Moscow Clin Res Ctr, Dept Upper Gastrointestinal Pancreat & Biliary Di, Moscow, Russia
[13] Ludwig Maximilian Univ Munich, Univ Hosp, Dept Med 2, Munich, Germany
[14] Univ Leipzig, Med Ctr, Med Dept Oncol Gastroenterol Hepatol Pulmonol Inf, Div Gastroenterol, Leipzig, Germany
[15] Ludwig Maximilian Univ Munich, Univ Hosp, Dept Gen Visceral & Transplantat Surg, Munich, Germany
[16] Palacky Univ, Univ Hosp Olomouc, Fac Med & Dent, Dept Internal Med Gastroenterol & Geriatr 2, Olomouc, Czech Republic
[17] Masaryk Univ, Univ Hosp Brno, Fac Med, Dept Surg, Brno, Czech Republic
[18] Masaryk Mem Canc Inst, Dept Gastroenterol & Digest Endoscopy, Brno, Czech Republic
[19] Karolinska Univ Hosp, Dept Upper Abdominal Dis, Stockholm, Sweden
[20] Univ Liverpool, Inst Translat Med, Dept Mol & Clin Canc Med, Liverpool, Merseyside, England
[21] Heidelberg Univ, Univ Med Ctr Mannheim, Med Fac Mannheim, Dept Med 2, Mannheim, Germany
[22] Univ Erlangen Nurnberg, Deutsch Zentrum Immuntherapie DZI, Dept Med 1, Kussmaul Campus Med Res, Erlangen, Germany
[23] Philipps Univ Marburg, Dept Gastroenterol & Endocrinol, Marburg, Germany
[24] Freeman Rd Hosp, HPB Unit, Newcastle Upon Tyne, Tyne & Wear, England
[25] Univ Sheffield, Dept Infect Immun & Cardiovasc Dis, Sheffield, S Yorkshire, England
[26] Univ Med Greifswald, Dept Med A, Greifswald, Germany
[27] Human Mater Domini, Gastrointestinal Endoscopy Unit, Castellanza, Italy
[28] Masaryk Univ, Univ Hosp Brno, Fac Med, Dept Pediat, Brno, Czech Republic
[29] Masaryk Univ, Cent European Inst Technol, Brno, Czech Republic
[30] Acibadem Mehmet Ali Aydinlar Univ, Sch Med, Istanbul, Turkiye
[31] Istanbul Univ, Istanbul Fac Med, Dept Gastroenterol, Istanbul, Turkiye
[32] Univ Vita Salute San Raffaele, IRCCS, San Raffaele Sci Inst, Pancreas Translat & Clin Res Ctr,Pancreato Biliar, Milan, Italy
[33] Univ Vita Salute San Raffaele, IRCCS, San Raffaele Sci Inst, Pancreas Translat & Clin Res Ctr,Div Pancreat Sur, Milan, Italy
[34] Univ Hosp Schleswig Holstein, Dept Internal Med 1, Kiel, Germany
[35] Univ Hosp, Dept Internal Med & Gastroenterol, Klinikum Oldenburg AoR, Oldenburg, Germany
[36] Univ Med Ctr Utrecht, Dept Gastroenterol & Hepatol, Utrecht, Netherlands
[37] Mediterranean Inst Transplantat & Adv Specialized, Dept Diagnost andTherapeut Serv, Endoscopy Serv, Palermo, Italy
[38] Univ Pecs, Inst Translat Med, Szentagotha Res Ctr, Med Sch, Pecs, Hungary
[39] Semmelweis Univ, Heart & Vasc Ctr, Div Pancreat Dis, Budapest, Hungary
[40] Masaryk Univ, Fac Med, Masaryk Mem Canc Ctr Inst, Dept Gastroenterol & Digest Endoscopy, Brno, Czech Republic
[41] IM Sechenov First Moscow State Med Univ, Moscow, Russia
[42] Tver State Med Univ, Dept Outpatient Therapy & Family Med, Tver, Russia
[43] Univ Duisberg Essen, Essen Univ Hosp, Dept Internal Med, Div Gastroenterol & Hepatol, Essen, Germany
[44] Univ Oxford, John Radcliffe Hosp, Nuffield Dept Med, Translat Gastroenterol Unit, Oxford, England
[45] Hosp Univ Cruces, Serv Aparato Digest, Biocruces, Grp Transplante Hepat, Baracaldo, Spain
[46] Univ Oslo, Fac Med, Campus Ahus, Oslo, Norway
[47] Martin Luther Univ Halle Wittenberg, Dept Internal Med 1, Halle, Germany
关键词
Autoimmune Pancreatitis; IgG4-Related Disease; IgG4-Related Pancreatitis; CONSENSUS DIAGNOSTIC-CRITERIA; INTERNATIONAL CONSENSUS; MULTICENTER; PREVALENCE; GUIDELINES; SUBTYPES; DISEASE;
D O I
10.1016/j.cgh.2023.12.010
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
BACKGROUND & AIMS: Autoimmune pancreatitis (AIP) is an immune -mediated disease of the pancreas with distinct pathophysiology and manifestations. Our aims were to characterize type 1 AIP in a large panEuropean cohort and study the effectiveness of current treatment regimens. METHODS: We retrospectively analyzed adults diagnosed since 2005 with type 1 or not -otherwisespeci fied AIP in 42 European university hospitals. Type 1 AIP was uniformly diagnosed using specific diagnostic criteria. Patients with type 2 AIP and those who had undergone pancreatic surgery were excluded. The primary end point was complete remission, defined as the absence of clinical symptoms and resolution of the index radiologic pancreatic abnormalities attributed to AIP. RESULTS: We included 735 individuals with AIP (69% male; median age, 57 years; 85% White). Steroid treatment was started in 634 patients, of whom 9 (1%) were lost to follow-up. The remaining 625 had a 79% (496/625) complete, 18% (111/625) partial, and 97% (607/625) cumulative remission rate, whereas 3% (18/625) did not achieve remission. No treatment was given in 95 patients, who had a 61% complete (58/95), 19% partial (18/95), and 80% cumulative (76/95) spontaneous remission rate. Higher (< 0.4 mg/kg/day) corticosteroid doses were no more effective than lower ( < 0.4 mg/kg/day) doses (odds ratio, 0.428; 95% confidence interval, 0.054 - 3.387) and neither was a starting dose duration > 2 weeks (odds ratio, 0.908; 95% confidence interval, 0.818 - 1.009). Elevated IgG4 levels were independently associated with a decreased chance of complete remission (odds ratio, 0.639; 95% confidence interval, 0.427 - 0.955). Relapse occurred in 30% of patients. Relapses within 6 months of remission induction were independent of the steroid -tapering duration, induction treatment duration, and total cumulative dose. CONCLUSIONS: Patients with type 1 AIP and elevated IgG4 level may need closer monitoring. For remission induction, a starting dose of 0.4 mg/kg/day for 2 weeks followed by a short taper period seems effective. This study provides no evidence to support more aggressive regimens.
引用
收藏
页码:994 / 1004
页数:11
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