Inflammatory bowel disease-like colitis pathology in a patient with common variable immune deficiency

Abstract

SUMMARYCommon variable immunodeficiency (CVID) can show variant histological patterns in the gastrointestinal system. We present a case of an 11-year-old boy who has been followed up with a diagnosis of CVID since he was 6 months old. He presented with abdominal pain and diarrhoea. Colonoscopic biopsy showed crypt destructive colitis, severe decrease and focal absence of plasma cells. Three months later he suffered from abdominal pain, vomiting and bloody diarrhoea. Macroscopic examination of small intestinal resection material revealed multiple perforation areas, ulcers. Histopathology showed mildmoderate active enteritis with aphthous ulcers, purulent peritonitis, decrease in plasma cells and loss of primary follicles in lymph nodes. Histopathological findings were consistent with inflammatory bowel disease (IBD)-like CVID. Although in 6-10% of patients with CVID an IBDlike presentation is observed, this highly aggressive form is rarely seen. We present this case because of its extraordinary presentation displaying perforating active enteropathy. BACKGROUND

Primary immunodeficiency and autoimmunity: A comprehensive review

“…In CVID patients, the most common ADs are cytopenias such as AIHA [174] and ITP [175]. Other ADs such as PA [176], SLE [177], JIA [178], T1D [179], VIT [180], and IBD [181,182] have also been reported. Azizi et al [183] did a retrospective cohort study that included 227 patients with CVID listed on the Iranian PID Registry.…”

Primary Humoral Immune Deficiencies: Overlooked Mimickers of Chronic Immune-Mediated Gastrointestinal Diseases in Adults

“…On the other hand, many authors are in favour of the theory that CVID enteropathy/colitis is a distinct independent disorder, separate from classic IBD, as there is an aforementioned lack of plasma cells in the mucosa [ 302 , 306 , 307 ]. Besides, this approach highlights that CVID can mimic lymphocytic colitis, collagenous colitis, and colitis associated with graft-versus-host disease [ 308 , 309 , 310 ].…”

“…The former includes classic IBD, specifically UC and CD. Non-crypt-destructive colitis comprises lymphocytic colitis and collagenous colitis [ 306 , 307 ]. Additionally, in CVID enteropathy/colitis, stricturing is observed; however, fistulation is not common [ 286 ].…”