Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective

Anne Jansen, Marcel van Deuren, Joanne Miller, Jiri Litzman, Javier de Gracia, Matías Sáenz-Cuesta, Anna Szaflarska, Timi Martelius, Yuichi Takiguchi, Smita Patel, Siraj Misbah, Anna Simon

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Abstract

© 2016 The Author(s) 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 60 years and median follow-up from onset of symptoms was 9 years. There was a high frequency of respiratory tract infections due to encapsulated bacteria. Median survival was 14 years. 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).
Original languageEnglish
Pages (from-to)12-17
JournalClinical Immunology
Volume171
DOIs
Publication statusPublished - 1 Oct 2016

Keywords

  • Good syndrome
  • Immunodeficiency
  • Infection
  • Thymoma

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    Jansen, A., van Deuren, M., Miller, J., Litzman, J., de Gracia, J., Sáenz-Cuesta, M., Szaflarska, A., Martelius, T., Takiguchi, Y., Patel, S., Misbah, S., & Simon, A. (2016). Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective. Clinical Immunology, 171, 12-17. https://doi.org/10.1016/j.clim.2016.07.025