DRKS-ID: DRKS00006025 Date of Registration in DRKS: 2014/03/26 Date of Registration in Partner Registry or other Primary Registry: [---]*

Trial Description Title A prospective study on clinical, serological and genetic factors of IgG4 - Related Disease Trial Acronym [---]*

URL of the trial [---]*

Brief Summary in Lay Language Immunoglobulin G4- related disease is a newly described systemic inflammatory disorder of unknown origin, characterized by tumor-like lesions, dense inflammatory infiltrates with IgG4- producing immune cells, tissue fibrosis and increased IgG4 serum levels. Manifestations have been described for nearly every organ system. Little is known about the origin of this rare disease and most studies focus on Asian populations. In this prospective study, we will establish an international and multi-centric cohort of 185 patients with IgG4- related disease and determine the HLA class II genotype of European patients with IgG4- related disease and healthy individuals to further elaborate on the origin of this orphan disease. Brief Summary in Scientific Language Immunoglobulin G4- related disease is a newly described systemic inflammatory disorder of unknown origin, characterized by tumefactive lesions, dense lymphoplasmacytic infiltrates with IgG4-positive plasma cells, storiform fibrosis and increased IgG4 serum levels. Manifestations have been described for nearly every organ system. Many diseases, that have been confined to single organs for a long time, have been recognized to be part of IgG4- related disease: Mikulicz's disease (dacroyoadenitis, sialadenitis), Küttner's tumor (sclerosing sialadenitis), Riedel's thyreoiditis, retroperitoneal fibrosis (Mb. Ormond), type1 autoimmune pancreatits and many others. Little is known about the initiation and pathophysiological processes involved in IgG4- related disease and most studies focus on Asian populations. There is evidence, that certain HLA alleles increase susceptibility to type 1 autoimmune pancreatitis in the Japanese population. Whether certain HLA alleles are linked to IgG4- related disease in European patients remains unknown. In this prospective study, we will establish an international and multi-centric cohort of 185 patients with IgG4- related disease and determine the HLA class II genotype of European patients with IgG4- related disease and healthy individuals to further elaborate on the origin of this orphan disease.

Organizational Data

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DRKS-ID: DRKS00006025 Date of Registration in DRKS: 2014/03/26 Date of Registration in Partner Registry or other Primary Registry: [---]*

DRKS-ID: DRKS00006025 Date of Registration in DRKS: 2014/03/26 Date of Registration in Partner Registry or other Primary Registry: [---]* Investigator Sponsored/Initiated Trial (IST/IIT): yes Ethics Approval/Approval of the Ethics Committee: Approved (leading) Ethics Committee Nr.: EK 13-238-1113 , Ethikkommission der Stadt Wien

Secondary IDs

Health condition or Problem studied ICD10: K85.01 - [generalization K85.0: Idiopathic acute pancreatitis]

Interventions/Observational Groups Arm 1: Patient cohort: Blood sampling for determination of possible HLA associations in IgG4-RD Arm 2: Control group (healthy people): Blood sampling to use as control for HLA associations.

Characteristics Study Type: Non-interventional Study Type Non-Interventional: Other Allocation: Other Blinding: [---]* Who is blinded: [---]* Control: Other Purpose: Basic research/physiological study Assignment: Other Phase: N/A Off-label use (Zulassungsüberschreitende Anwendung eines Arzneimittels): N/A

Primary Outcome HLA class II genotypes in IgG4-RD as compared to the general population,

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DRKS-ID: DRKS00006025 Date of Registration in DRKS: 2014/03/26 Date of Registration in Partner Registry or other Primary Registry: [---]*

Secondary Outcome Evaluation of single nucleotide polymorphisms (CTLA4, TNF-alpha, FCRL3), assessment of laboratory values of immunological relevance, molecular biological examination of cytokines and chemokines, analysis of autoantibody profiles, characterization of PBMC subpopulations, evaluation of demographic, clinical and quality of life parameters as determined by SF36.

Countries of recruitment AT Austria DE Germany IT Italy

Locations of Recruitment Medical Center Hanusch Krankenhaus, Wien University Medical Center Universitätsklinikum Graz, Graz Medical Center AKH Linz, Linz Medical Center Kaiser-Franz-Josef-Spital, Wien University Medical Center Erlangen University Medical Center Parma Medical Center Arcispedale Santa Maria Nuova, Reggio Emilia

Recruitment Planned/Actual: Actual (Anticipated or Actual) Date of First Enrollment: 2014/03/27 Target Sample Size: 370 Monocenter/Multicenter trial: Multicenter trial National/International: International Inclusion Criteria Gender: Both, male and female Minimum Age: 18 Years Maximum Age: 75 Years

Additional Inclusion Criteria

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DRKS-ID: DRKS00006025 Date of Registration in DRKS: 2014/03/26 Date of Registration in Partner Registry or other Primary Registry: [---]*

Definite, probable or possible diagnosis of IgG4-RD according to published criteria (Umehara H et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012 Feb;22(1):21-30) and exclusion of differential diagnoses

Exclusion criteria IgG4-RD not confirmed

Addresses Primary Sponsor Ludwig Boltzmann-Institut für Osteologie im Hanusch-Krankenhaus der WGKK und Unfallkrankenhaus Meidling der AUVA, 1. Medizinische Abteilung, Hanusch-Krankenhaus Mr. Prim. Univ.-Prof. Klaus Klaushofer Heinrich-Collin-Straße 30 1140 Wien Austria Telephone: +43 1 91021 86921 Fax: +43 1 91021 86929 E-mail: klaus.klaushofer at osteologie.at URL: [---]* Contact for Scientific Queries Ludwig Boltzmann-Institut für Osteologie im Hanusch-Krankenhaus der WGKK und Unfallkrankenhaus Meidling der AUVA, 1. Medizinische Abteilung, Hanusch-Krankenhaus Mr. PD Dr. Jochen Zwerina Heinrich-Collin-Straße 30 1140 Wien Austria Telephone: +43 1 91021 86921 Fax: +43 1 91021 86929 E-mail: jochen.zwerina at osteologie.at URL: [---]* Contact for Public Queries Ludwig Boltzmann-Institut für Osteologieim Hanusch-Krankenhaus der WGKK undUnfallkrankenhaus Meidling der AUVA1. Medizinische Abteilung, HanuschKrankenhaus Mr. PD Dr. Jochen Zwerina Heinrich-Collin-Straße 30 1140 Wien Austria

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DRKS-ID: DRKS00006025 Date of Registration in DRKS: 2014/03/26 Date of Registration in Partner Registry or other Primary Registry: [---]*

Contact for Public Queries Ludwig Boltzmann-Institut für Osteologieim Hanusch-Krankenhaus der WGKK undUnfallkrankenhaus Meidling der AUVA1. Medizinische Abteilung, HanuschKrankenhaus Mr. PD Dr. Jochen Zwerina Heinrich-Collin-Straße 30 1140 Wien Austria Telephone: +43 1 91021 86921 Fax: +43 1 91021 86929 E-mail: jochen.zwerina at osteologie.at URL: [---]*

Sources of Monetary or Material Support Commercial (pharmaceutical industry, medical engineering industry, etc.) Roche Pharma 1210 Wien Austria Telephone: [---]* Fax: [---]* E-mail: [---]* URL: [---]* Public funding institutions financed by tax money/Government funding body (German Research Foundation (DFG), Federal Ministry of Education and Research (BMBF), etc.) Medizinisch-Wissenschaftlicher Fonds des Bürgermeisters der Bundeshauptstadt Wien Thomas-Klestil-Platz 8 1030 Wien Austria Telephone: [---]* Fax: [---]* E-mail: [---]* URL: [---]*

Status Recruitment Status: Recruiting ongoing Study Closing (LPLV): [---]*

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DRKS-ID: DRKS00006025 Date of Registration in DRKS: 2014/03/26 Date of Registration in Partner Registry or other Primary Registry: [---]*

Trial Publications, Results and other documents

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