Sprue-Like and Other Intestinal Diseases in the Common Variable Immunodeficiency (Cvid) Spectrum

Many forms of immunodeficiency have been described. There may be about 150 different types, some with inherited or genetic causes, some with recognized specific molecular defects. Most can be classified as affecting the humoral or cellular, sometimes both, immune systems. In adults, the most common symptomatic form, albeit rare, is common variable immunodeficiency, or CVID. In recent years, it has also been suggested that rather than a single disease, CVID represents a heterogeneous group marked by panhypogammaglobulinemia and variable clinical features. In most, intestinal symptoms predominate, including, but not limited to sprue-like intestinal disease, a disorder that fails to respond to a gluten-free diet. Also, several types of colonic disease may occur similar to ulcerative colitis, Crohn’s disease and, most recently reported, collagenous colitis. Treatment of these disorders remains largely empirical and based on disease seen in the absence of CVID. Added molecular genetic studies are needed to more fully characterize the immune defects in CVID and, ultimately, to provide more evidence-based treatments.