Background: Common variable immunodeficiency (CVID) is a heterogeneous group of primary immune deficiency disorders that may be characterized by heightened susceptibility to gastrointestinal (GI) infection. GI conditions manifest in 20 to 50% of CVID patients but rarely include cholangitis.
Methods/Results: This is a 61-year-old female, with a history of cholecystectomy and gastric bypass, who presented with recurrent ascending cholangitis for eight years. After a hepaticojejunostomy to correct a bile duct stricture complication from the cholecystectomy, ascending cholangitis was diagnosed by clinical presentation of fever and right upper quadrant pain and imaging revealing pneumobilia. CVID was diagnosed after no response to pneumococcal polysaccharide vaccine, as well as serum IgA and IgG measured below the normal ranges. The patient was prescribed a successful, weekly regimen of 15 g of oral intravenous immunoglobulin (IVIG) ten percent liquid (Gammaplex® 10%, 5 gm/50 mL).
Conclusion: Oral human IVIG is a novel treatment and has been infrequently utilized for management of chronic rotavirus, necrotizing enterocolitis, diarrhea, HIV enteropathy, irritable bowel syndrome, and malnutrition. This is the first case of recurrent ascending cholangitis as the primary manifestation of CVID, as well as successful treatment of this condition with oral IVIG, in the literature.