title: Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective.
abstract: Good syndrome (GS) or thymoma-associated immunodeficiency, is a rare condition that has only been studied in retrospective case series. General consensus was that GS has a worse prognosis than other humoral immunodeficiencies. In this study, physicians of GS patients completed two questionnaires with a two year interval with data on 47 patients, 499 patient years in total. Results on epidemiology, disease characteristics, and outcome are presented. Mean age at diagnosis was 60years and median follow-up from onset of symptoms was 9years. There was a high frequency of respiratory tract infections due to encapsulated bacteria. Median survival was 14years. Survival was reduced compared to age-matched population controls (5-year survival: 82% versus 95%, p=0.008). In this cohort survival was not associated with gender (HR 0.9, 95% CI 0.3-3.0), autoimmune diseases (HR 2.9, 95% CI 0.8-10.1) or immunosuppressive use (HR 0.3, 95% CI: 0.1-1.2).
authors: Nijmegen Center for Immunodeficiency and Autoinflammation (NCIA), Department of Internal Medicine, Radboud University Medical Centre, Nijmegen, The Netherlands. Electronic address: Anne.FM.Jansen@radboudumc.nl.
authors: Anne
authors: A
authors: Jansen
authors: Nijmegen Center for Immunodeficiency and Autoinflammation (NCIA), Department of Internal Medicine, Radboud University Medical Centre, Nijmegen, The Netherlands.
authors: Marcel
authors: M
authors: van Deuren
authors: Department of Immunology, John Radcliffe Hospital, Oxford, United Kingdom. Electronic address: Joanne.Miller@ouh.nhs.uk.
authors: Joanne
authors: J
authors: Miller
authors: Department of Clinical Immunology and Allergology, Faculty of Medicine, Masaryk University, St Anne's University Hospital, Brno, Czech Republic. Electronic address: litzman@fnusa.cz.
authors: Jiri
authors: J
authors: Litzman
authors: Department of Pulmonary Diseases, Universitat Autònoma de Barcelona, CIBER Ciberes. Barcelona, Spain. Electronic address: jgracia@vhebron.net.
authors: Javier
authors: J
authors: de Gracia
authors: Donostia University Hospital, Biodonostia Health Research Institute, San Sebastian, Spain. Electronic address: matiasbernabe.saenzcuesta@osakidetza.net.
authors: Matías
authors: M
authors: Sáenz-Cuesta
authors: Department of Clinical Immunology and Transplantology, Jagiellonian University, Medical College and Children University Hospital, Cracow, Poland. Electronic address: miszafla6@wp.pl.
authors: Anna
authors: A
authors: Szaflarska
authors: Department of Infectious Diseases, University of Helsinki and Helsinki University Hospital, Helsinki, Finland. Electronic address: timi.martelius@hus.fi.
authors: Timi
authors: T
authors: Martelius
authors: Department of Medical Oncology, Chiba University, Chiba, Japan. Electronic address: takiguchi@faculty.chiba-u.jp.
authors: Yuichi
authors: Y
authors: Takiguchi
authors: Department of Immunology, John Radcliffe Hospital, Oxford, United Kingdom.
authors: Smita
authors: S
authors: Patel
authors: Department of Immunology, John Radcliffe Hospital, Oxford, United Kingdom.
authors: Siraj
authors: S
authors: Misbah
authors: Nijmegen Center for Immunodeficiency and Autoinflammation (NCIA), Department of Internal Medicine, Radboud University Medical Centre, Nijmegen, The Netherlands.
authors: Anna
authors: A
authors: Simon
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000000981:diagnostic imaging / Q000453:epidemiology; D002648:Child; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000000981:diagnostic imaging / Q000453:epidemiology*; D007239:Infections / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011795:Surveys and Questionnaires; D013945:Thymoma / Q000000981:diagnostic imaging / Q000453:epidemiology*; D013953:Thymus Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology*
keywords: Good syndrome; Immunodeficiency; Infection; Thymoma