Panhypogammaglobulinemia associated with bronchiectasis in the present case are classic findings of CVID that explain the repeated episodes of pneumonia. Unresponsiveness to diarrhea was initially thought to be a distal UC, which, unusually, was hard to control; however, the almost normal features on endoscopic (colonoscopy) and histological controls associated with the rapid and sustained positive response after the first gamma globulin infusion, allowing the withdrawal of the steroid and azathioprine, suggest that the gastrointestinal disorders were probably caused by the underlying overlap immunodeficiency process.
Besides the present case, we also have 2 others with CVID among Ο·150 patients whose cases we have followed in our IBD outpatient unit. One of them has ileocolitis CD without symptoms of immunodeficiency: the diagnosis was made just by chance during immunological screening. The second patient was initially diagnosed with classic CIVD and was kept on regular gammaglobulin infusions. Later, the investigation of gastrointestinal symptoms resulted in the detection of histological IC. More recently he has developed liver and biliary enzyme elevations (primary sclerosing cholangitis?), as well as splenomegaly and mesenteric lymph nodes (lymphoma?) that are under investigation.
These cases, particularly the 1 described in depth here, highlight the need to be aware of the possibility of immunodeficiency disorders in IBD patients (as the cause or as a consequence, we do not know yet), who may develop unusual responses to common therapeutic applications, or an overlap process of recurrent infections, or any autoimmune disease, or cancer.