The ESID Online Database

EUROPEAN SOCIETY FOR IMMUNODEFICIENCIES The ESID Online Patient- & Research Database The ESID online database Progress Report, FDA, 25 April 2007 Report, FDA, 25 April 2007 www.esid.org Bodo Grimbacher, MD ESID Secretary Royal Free Hospital & University College London Medical School, Dep. of Immunology & Molecular Pathology b.grimbacher@medsch.ucl.ac.uk Current status of Documenting Centres Current status of Documenting Centres Statistics Statistics 4500 4000 Number of patients, April 2007: 4,142 3500 3000 2500 2000 1500 1000 500 0 Apr. 05: 231 Apr. 05: 231 Jun 05: 500 Jun 05: 500 Dec 05: 1183 Dec 05: 1183 Jun 06: 2148 Jun 06: 2148 Apr 07: 4142 Apr 07: 4142 Follow-up documentation Follow-up documentation 70% 60% 57.98% 50% No of patients 40% 30% 21.83% 20% 10% 9.84% 3.75% 1.35% 0.51% 6 0.46% 7 0.48% 8 0.27% 9 0.24% 10 >10 3.29% 0% 1 2 3 4 5 Number No of visit dates of documented visits Statistics Statistics Predominantly antibody disorders 59% Other well defined PIDs 17% Phagocytic disorders 14% Predominantly T-cell deficiencies 6% Autoinflammatory syndromes 0% Autoimmune and immunedysregulation syndromes 1% Unclassified Immunodefieciencies 2% Complement deficiencies 1% The ESID Online Patient and Research Database The ESID Online Patient and Research Database Background • clinical online database, accessible via standard internet browser, hosted in Freiburg, Germany • online since August 2004 • personalized or pseudonymized version available • covers all 206 PIDs with a common core-dataset • has 29 disease-specific extended sub-registries • continued documentation of patients • mutation documentation linked to public mutation databases • percentile curves • SF 36 questionnaire for QoL monitoring • predefined queries • sponsored by five PPTA member companies and the EU Subregistries online Subregistries online Disease-specific data models available for... variable immunodeficiency • Agammaglobulinemias including XLA • Selective IgA deficiency • IgG subclass deficiency • Ataxia Teleangiectasia • DiGeorge Syndrome • Hyper IgE Syndrome • Nijmegan Breakage syndrome • Specific IgG Deficiency • Secondary hypogammaglobulinemia • Osteopetrosis • ICF Syndrome • Comel Netherton Syndrome • IPEX • Common …and many others Immunoglobulin Therapy Immunoglobulin Therapy Total number of patients on Ig replacement: 1,741 subcutanous Ig 321 18% intravenous Ig 1361 79% unknown 49 3% intramuscular Ig 4 0% oral Ig 6 0% Frequency of infections in PID patients in relation to Frequency of infections in PID patients in relation to treatment with immunoglobulin replacement treatment with immunoglobulin replacement Quality of life queries (2006) Quality of life queries (2006) First Publication First Publication Clinical and Experimental Immunology, 147: 306–312, 2007 Second Publication Second Publication The ESID Online Patient and Research Database The ESID Online Patient and Research Database Summary • online database suitable to answer clinical and research questions • runs stable since August 2004 • contains data of > 4,130 patients • covers all 206 PIDs with a common core-dataset • has 29 disease-specific extended registries for specific studies • continued follow-up documentation on patients • is ready to address clinical studies including post licensing surveillance • can be easily adapted to other disease entities (XML programming) • (e.g. rheumatic, infectious, cardiologic, and cancer) Contact: Bodo Grimbacher, ESID Secretary: b.grimbacher@medsch.ucl.ac.uk Bodo Grimbacher, MD, ESID Secretary Dept. of Immunology & Molecular Pathology - University College London, Royal Free Hospital, Pond Street, London NW3 2QG, UK Tel.: +44-20-7794 0500 ext 34399 Fax: +44-207 433 1943 E-mail: b.grimbacher@medsch.ucl.ac.uk funded by: