Differences in Clinical Outcome in Patients with Common Variable Immunodeficiency Treated with Ig Replacement Therapy: Results from the ESID Database

Abstract

S A T U R D A Y 36 Differences in Clinical Outcome in Patients with Common Variable Immunodeficiency Treated with Ig Replacement Therapy: Results from the ESID Database G. Kindle, K.Warnatz, O. Paschenko, D. Kumararatne, S. S. Kilic, V. Thon, T. Witte, M. Helbert, T. W. Kuijpers, A. Exley, N. Mahlaoui, G. Notheis, H. Longhurst, U. Baumann, A. Jones, N. K€ut€ukc€uler, M. Borte, P. Wagstr€om, C. Feighery, A. Szaflarska, H. Ritterbusch, S. Reda, T. Kononova, Z. Panahloo, B. Grimbacher; Centre of Chronic Immunodeficiency (CCI), University Medical Center Freiburg and University of Freiburg, Freiburg, GERMANY, Russian Children’s Clinical Hospital, Moscow, RUSSIAN FEDERATION, Cambridge University Hospital NHS Trust (Addenbrooke’s), Cambridge, UNITED KINGDOM, Uludag University Medical Faculty, Bursa, TURKEY, Masaryk University Medical Faculty, St Anne University Hospital, Brno, CZECH REPUBLIC, Department of Clinical Immunology, Hannover Medical School, Hannover, GERMANY, University of Manchester, Manchester, UNITED KINGDOM, Emma Children’s Hospital, Academic Medical Center (AMC), Amsterdam, NETHERLANDS, Cambridge University Health Partners, Papworth Hospital NHS Foundation Trust, Cambridge, UNITED KINGDOM, CEREDIH: The French PID study group, Unit e d’Immuno-H ematologie & Rhumatologie p ediatriques, Hopital Necker-Enfants Malades, Paris, FRANCE, Dr v. Haunersches Kinderspital, Ludwig Maximilians University, Munich, GERMANY, Barts and the London NHS Trust, London, UNITED KINGDOM, Paediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, GERMANY, Institute of Child Health/Great Ormond Street Hospital, London, UNITED KINGDOM, Ege University, Faculty of Medicine, Dept of Pediatric Immunology, Bornova, Izmir, TURKEY, Children’s Hospital, Municipal Hospital ’St Georg’, Academic Teaching Hospital of the University of Leipzig, Leipzig, GERMANY, Ryhov County Hospital, Jonkoping, SWEDEN, Department of Immunology,Trinity College Dublin & St. James’ Hospital, Dublin, IRELAND, Department of Clinical Immunology, PolishAmerican Institute of Paediatrics, Medical College, Jagiellonian University, Cracow, POLAND, Department of Pediatric Allergy and Immunology, Children’s Hospital, Faculty of Medicine, Ain Shams University, Cairo, EGYPT, Research and Clinical Center for Pediatric Hematology, Oncology and Immunology, Moscow, RUSSIAN FEDERATION, Medical Science Department, CSL Behring, Haywards Heath, UNITED KINGDOM, Royal Free Hospital & University College London, London, UNITED KINGDOM. RATIONALE:Themost frequent primary immunodeficiency diagnosis in the ESID Database is common variable immunodeficiency (CVID; n52401). The primary treatment is immunoglobulin (Ig) replacement therapy by intravenous (IV) or subcutaneous (SC) administration. We retrospectively assessed the clinical outcome with Ig therapy in patients with CVID by country and route of administration. METHODS:Datawere provided for patients with CVIDwhowere treated with Ig therapy. Four health parameters were evaluated by country and by route of administration. The number of patients with available data varied from 417 to 645 between the four parameters. RESULTS: There was a highly significant variation between countries in the four outcomes (median occurrence per year, p<0.001). The number of days unable to perform daily duties was highest in Poland (22.5) and Netherlands (7.0), and lowest in the UK (1.1). The number of days in hospital due to the immunodeficiency was highest in Poland (18.3) and Turkey (10.7). The overall number of infections was lowest in Russia and Sweden (both 1.0) and highest in Turkey (6.0). Turkey also had the highest number of serious bacterial infections (2.0). In contrast, the differences observed in these clinical outcomes between IVIg versus SCIg therapy were relatively small. CONCLUSIONS: These results appear to show a wide regional variation, whereas the differences observed between IVand SC administration were relatively small. Further research into the confounding factors such as the socio-economic impact of sick-leave, available treatment options in the respective countries and existing local CVID management protocols, should be conducted.