1. Prevalence and clinical challenges among adults with primary immunodeficiency and recombination-activating gene deficiency 
Dylan Lawless, MSc, Christoph B. Geier, BSc, Jocelyn R. Farmer, MD, PhD, Hana Lango Allen, PhD, Daniel Thwaites, MSc, Faranaz Atschekzei, MD, PhD, Matthew Brown, BSc, David Buchbinder, MD, Siobhan O. Burns, MD, PhD, Manish J. Butte, MD, PhD, Krisztian Csomos, PhD, Sri V.V. Deevi, PhD, William Egner, MD, PhD, Stephan Ehl, MD, Martha M. Eibl, MD, Olajumoke Fadugba, MD, Zsofia Foldvari, MD, Deanna M. Green, MD, Sarah E. Henrickson, MD, PhD, Steven M. Holland, MD, Tami John, MD, Christian Klemann, MD, Taco W. Kuijpers, MD, PhD, Fernando Moreira, BSc, Alexander Piller, MSc, Paula Rayner-Matthews, BSc, Neil D. Romberg, MD, Ravishankar Sargur, MD, Reinhold E. Schmidt, MD, Claudia Schröder, PhD, Catharina Schuetz, MD, Svetlana O. Sharapova, PhD, Ken G.C. Smith, MD, PhD, Georgios Sogkas, MD, Carsten Speckmann, MD, Kathleen Stirrups, PhD, Adrian J. Thrasher, MD, PhD, Hermann M. Wolf, MD, Luigi D. Notarangelo, MD, Rashida Anwar, PhD, Joan Boyes, PhD, Boglarka Ujhazi, MSc, James Thaventhiran, MD, PhD, Jolan E. Walter, MD, PhD, Sinisa Savic, MD, PhD 
Journal of Allergy and Clinical Immunology 
Volume 141, Issue 6, Pages (June 2018)
DOI: /j.jaci
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2. Fig 1
Gene variants and phenotypes of adults with RAG deficiency. A, Schematic representation of RAG1 and RAG2 adapted from Notarangelo et al.E19 Variants in this cohort (17 in RAG1 and 8 in RAG2) are shown in red. Known pathogenic variants reviewed previously are shown as blue dots.E19 B, Phenotypes of RAG deficiency in 15 patients, including those from the National Institute for Health Research BioResource–Rare Diseases PID and Vienna cohorts. C, Presentation of lung disease (n = 15). CID, Combined immunodeficiency; CID G/AI, combined immunodeficiency with granuloma and/or autoimmunity; COPD, chronic obstructive pulmonary disease; CVID, common variable immunodeficiency; ICL, idiopathic CD4+ T lymphopenia; SPAD, specific polysaccharide antibody deficiency.
Journal of Allergy and Clinical Immunology  , DOI: ( /j.jaci )
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3. Fig 2
Pulmonary disease. A, Onset of pneumonia, bronchiectasis, and granuloma/fibrosis/chronic obstructive pulmonary disease (COPD; n = 15). B, High-resolution computed tomography of patients 3 and 9. Histologic examination of lung biopsy specimens from patient 1 with atypical lymphoid hyperplasia with granuloma and fibrosis (hematoxylin and eosin).
Journal of Allergy and Clinical Immunology  , DOI: ( /j.jaci )
Copyright © 2018 The Authors Terms and Conditions