Common variable immunodeficiency (CVID) is associated with gastrointestinal manifestations, in particular diarrhea (1,2). It is linked to autoimmune disorders such as nodular lymphoid hyperplasia (NLH), possibly increasing the risk of lymphoma (3). The prevalence of chronic inflammatory bowel disease (CIBD) in these patients is increased, especially Crohn's disease (4,5), and its treatment does not seem to differ from the standard (2). We present a patient with CVID and ulcerative colitis treated in the conventional manner.
Case reportA 45-year-old male with CVID and intravenous immunoglobulin replacement diagnosed 7 years ago with ulcerative colitis and treated with mesalazine.Two years ago, he presented diarrhea with pathological content, little response to oral corticosteroids. Colonoscopy was normal. After ruling out other causes of diarrhea, we carried out a capsule endoscopy which showed marked NLH of the small intestine. The clinical presentation was considered as secondary to the CVID.Fourteen months ago, we observed clinical worsening, elevated calprotectin and a colonoscopy with pancolitis and moderate activity. Due to dependence on corticosteroids, we started azathioprine, presenting with clinical improvement and decrease in calprotectin serum levels, but with hepatotoxicity, which also occurred when changing to mercaptopurine. We initiated methotrexate, which was well tolerated but produced hepatotoxicity. Given the persistence of the clinical presentation and endoscopic activity, we started adalimumab 7 months ago, with good tolerance, clinical response and serum levels of calprotectin almost returning to normal.
DiscussionCases of CIBD treated in a conventional manner, including biologics (4-6), have been published. It is believed that inhibition of TNFα by these also improves CVID symptoms, which increases the levels of TNFα (5). We were presented with one of the few described cases of ulcerative colitis and CVID treated with thiopurines, methotrexate and anti-TNFα. It must be emphasized the difficult diagnosis of diarrhea in these patients and the usefulness of classic treatments, since we have shown a good response without complications derived from CVID. In our opinion, the treatment should not be different if there is correct replacement of immunoglobulins.