Anti-tumour necrosis factor-alpha therapy for severe enteropathy in patients with common variable immunodeficiency (CVID)

Abstract

We present three common variable immunodeficiency (CVID) patients with severe inflammatory bowel disease of unknown aetiology, resistant to steroid treatment, treated with infliximab. After exclusion of any infection, infliximab was given at a dose of 5 mg/kg every 4 weeks for a 3 month induction followed by every 4-8 weeks depending on clinical response. Two of these patients had predominantly small bowel disease; they both showed clinical response to infliximab with weight gain and improvement of quality of life scores. The third patient had large bowel involvement with profuse watery diarrhea; this patient improved dramatically within 48 hours of having infliximab treatment. All three patients have been maintained on infliximab treatment for between 5 and 53 months (mean 37 months) with no evidence of increased susceptibility to infections in the patients with small bowel disease, although the third patient developed two urinary tract infections and a herpes zoster infection following therapy. This is the first small case series to show that infliximab is a useful addition to current therapy in this rare group of patients with potentially life threatening enteritis.

Fig. 1

Patient 1 weight response to infliximab with symbols (♦) indicating infliximab infusions. Deterioration at 40 months was due to campylobacter infection.

Fig. 2

Inflammatory Bowel Disease Questionaire scores for the three patients with markers representing infliximab infusion. The scoring was started in patient 1 after she had already had nine treatments of infliximab.

Fig. 3

Patient 3 bowel frequency response to a single infliximab infusion.

Fig. 4

Small and large bowel inflammation of patient 1 (before infliximab). (a) Low (× 25 magnification) and (b) high (× 200 magnification) power images of the duodenal biopsy show features similar to coeliac disease, with flattened villi, crypt hyperplasia and an increase in lamina propria chronic inflammatory cell infiltrate and epithelial lymphocytes. (c) Low (× 50 magnification) and (d) high (× 100 magnification) power images of the colon biopsy show mild crypt distortion and a patchy chronic inflammatory cell infiltrate. Focal active inflammation (cryptitis) is demonstrated in the top left gland on higher power (d).