D. Ravindran, RSR. Mugunthan, TK. Shruthi and D. Jayaraman (2022). Severe diabetic ketoacidosis and autoimmune pancreatitis with SIRS in an adolescent with LRBA deficiency - A rare complication of a common primary immunodeficiency disease. Journal of family medicine and primary care, 11(4), 1552-1554. https://doi.org/10.4103/jfmpc.jfmpc_1220_21

PubMed 35516664

[Abstract]
Common variable immunodeficiency is the most common primary immunodeficiency disorder. Lipopolysaccharide (LPS)-responsive beige-like anchor protein (LRBA) deficiency is categorized as a common variable immunodeficiency associated with autoimmune manifestations and inflammatory bowel diseases. We report a rare case, an adolescent presenting with severe diabetic ketoacidosis (DKA) and acute pancreatitis with multiorgan dysfunction with common variable immunodeficiency (CVID) with homozygous LRBA mutation.