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. 2020 Oct 21;11:574738. doi: 10.3389/fimmu.2020.574738

Figure 1.

Figure 1

Demographic and clinical characterization patients with vasculitis due to RAG deficiency. (A) Vasculitis is the fourth most common complication of pRD with immune dysregulation in a cohort of 62 patients (modified from 13) (B) Clinical diagnosis of PID in years compared between patients with pRD (n = 8, circles = severe/multiorgan, rectangle = mild/skin-only) or without (n = 54) vasculitis and RAG deficiency (*p < 0.05) (C) Percent of patients alive by age and annotate clinical milestones (D) Kaplan-Meier curves comparing survival of RAG-deficient patients with (n = 8, dotted line) and without (n = 54, straight line) vasculitis (E) Overall frequency of autoimmune complications besides vasculitis in adult patients with RAG deficiency, AIC… autoimmune cytopenia, AI… autoimmunity (F) Recombination activity from all available RAG1/2 alleles (average of % wild-type protein). For the non-vasculitis control group only patients with CID-G/AI and AS phenotype were considered. (circles = severe/multiorgan, rectangle = mild/skin-only) (ns statistically not significant, *p ≤ 0.05, ***p ≤ 0.001).