CTLA4 and Autoimmunity: In addition to the impaired suppressive function of Tregs, which is at least partially explained by the reduction in CTLA-4 expression,59 the majority of LRBA-deficient patients display reduced Treg counts.60 Autoimmunity is reported to be the most common manifestation of LRBA deficiency and includes enteropathy, autoimmune cytopenias, endocrinopathy, interstitial lung disease, and/or autoimmune hepatitis.