Clinical Experience of CVID Enteropathy
Sunday, March 6, 2016: 2:15 PM
Room 408A (Convention Center)
Edith Schussler, MD, , ,
Rationale: Gastrointestinal symptoms are common in CVID patients and often severe.  We report our experience of CVID enteropathy.

Methods: We examined the clinical and pathological findings of CVID patients at our institution with enteropathy in a retrospective chart review.

Results: 53 CVID patients with enteropathy were identified. 29 were female and 24 male.  Mean age at diagnosis of CVID was 32 years (median 32 range 2-74 years), mean IgG 205 (median 210, range 0-641), mean IgA 13 (median 6, 0-155 ) , mean IgM 29 (median 14, range 0-400), mean peripheral lymphocyte subsets were 69.7 % T (median 73%) and 8.3% B (median 9%).  23.6% of these patients reported autoimmune disease at the time of diagnosis. Three subjects had previously had stomach, breast and testicular cancer. 20% were diagnosed with clinically significant malabsorption and 5 were on TPN at some point during their course. Of the 55 patients, 17 had died at the time of review.  Mean age of death was 44 (median 42, range 22-77).  Median years from diagnosis to death was 11 (median 10, range 1-29).   We identified a subset of 10 patients treated with budesonide for severe GI symptoms. 7/10 of these patients showed improvement measured as reported frequency of bowel movements.

Conclusions: CVID enteropathy is associated with severe morbidity and mortality.  Use of oral budesonide can have beneficial effects on the frequency of bowel movement in patients with severe CVID enteropathy, however additional and more effective treatments are needed.