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. 2018 May 14;9:694. doi: 10.3389/fimmu.2018.00694

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Copyright © 2018 Slade, Bosco, Binh Giang, Kruse, Stirling, Cameron, Hore-Lacy, Sutherland, Barnes, Holdsworth, Ojaimi, Unglik, De Luca, Patel, McComish, Spriggs, Tran, Auyeung, Nicholls, O’Hehir, Hodgkin, Douglass, Bryant and van Zelm.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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CVID is associated with more frequent non-infectious complications than other predominantly antibody deficiencies, and these complications are associated with reduced survival. (A) Histogram depicting frequency of complications in CVID, darker-colored columns, compared with non-CVID cases in lighter-colored columns. Abbreviations: GLILD, granulomatous lymphocytic interstitial lung disease; CVID, common variable immunodeficiency. Purple represents bronchiectasis; yellow represents non-malignant lymphoproliferative complications; brown, autoimmune complications; green, gastrointestinal disease, and blue, malignancies. (Fisher’s exact test: ***p < 0.001; **p < 0.01; and *p < 0.05). (B) Histogram depicting percentage of CVID and non-CVID patients with 0, 1, or more non-infectious complications of disease. (C) Survival after diagnosis in individuals with immune dysregulation is significantly reduced compared with patients with infections only (p < 0.05, Mantel–Cox log rank). Black line indicates survival of individuals without features of immune dysregulation, red line indicates survival of individuals with immune dysregulation. (D) Survival after diagnosis in individuals with bronchiectasis is significantly reduced compared with patients with infections only (p < 0.05, Mantel–Cox log rank). Black line indicates survival of individuals without bronchiectasis, and red indicates survival of individuals with bronchiectasis.