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. Author manuscript; available in PMC: 2019 Jul 7.
Published in final edited form as: J Allergy Clin Immunol Pract. 2019 Mar 12;7(6):1970–1985.e4. doi: 10.1016/j.jaip.2019.02.038

Figure 7. A spectrum of other autoimmune and hyperinflammatory diseases occur in RAG deficiency.

Figure 7

(A) Prevalence of single- and multi-organ granulomas listed by anatomic location (frequency as % total cases). (B) Age of onset for the other autoimmune and hyperinflammatory complications (dots representing individual patients, median +/− 95% CI shown, clinical milestones annotated). (C) Prevalence of positive autoantibodies (frequency as % total cases). (D) Granuloma treatment response, scored by individual treatment modality for each incidence of granulomatous disease (% response per trialed therapeutic shown by color gradation as indicated; therapeutic grouping by first-line (IVIG, steroids, and/or anti-infectives), second-line (all biologics), and third-line (HSCT) agents as shown; number of annotated therapeutic trials shown); acetylcholine receptor (AChR); anti-mitochondrial antibody (AMA); anti-nuclear antibody (ANA); anti-phospholipid antibody (APLA); cyclosporin A (CsA); double stranded DNA (dsDNA); glutamic acid decarboxylase (GAD); hematopoietic stem cell transplant (HSCT); intravenous immunoglobulin (IVIG); methotrexate (MTX); mycophenolate mofetil (MMF); rheumatoid factor (RF).