Adult Celiac Disease and Its Malignant Complications

Abstract

Adult celiac disease is a chronic intestinal disorder that has been estimated to affect up to 1-2% of the population in some nations. Awareness of the disease has increased, but still it remains markedly underdiagnosed. Celiac disease is a pathologically defined condition with several characteristic clinical scenarios that should lead the clinician to suspect its presence. Critical to diagnosis is a documented responsiveness to a gluten-free diet. After diagnosis and treatment, symptoms and biopsy-proven changes may recur and appear refractory to a gluten-free diet. Recurrent symptoms are most often due to poor diet compliance, a ubiquitous and unrecognized gluten source, an initially incorrect diagnosis, or an associated disease or complication of celiac disease. Some patients with persistent symptoms and biopsy-proven changes may not have celiac disease at all, instead suffering from a sprue-like intestinal disease, so-called unclassified sprue, which is a specific entity that does not appear to respond to a gluten-free diet. Some of these patients eventually prove to have an underlying malignant cause, particularly lymphoma. The risk of developing lymphoma and other malignancies is increased in celiac disease, especially if initially diagnosed in the elderly, or late in the clinical course of the disease. However, recent studies suggest that the risk of gastric and colon cancer is low. This has led to the hypothesis that untreated celiac disease may be protective, possibly due to impaired absorption and more rapid excretion of fat or fat-soluble agents, including hydrocarbons and other putative cocarcinogens, which are implicated in the pathogenesis of colorectal cancer.

Sprue-like Intestinal Disease

“…In general, lymphomas of B-cell lineage affect the small intestine most commonly, while T-cell lymphomas are less common. Lymphomas are notoriously very heterogeneous [26,27,28]. Some may complicate well-defined celiac disease, but some may not [26,27].…”

“…Lymphomas are notoriously very heterogeneous [26,27,28]. Some may complicate well-defined celiac disease, but some may not [26,27]. For example, a natural killer cell form of T-cell lymphoma may occur in the intestine with a distinct immunophenotype.…”

Celiac Disease and Cancer – Are There Potential Links? Is the Vigilance of Immune System in Celiac Disease a Double-edged Sword?

“…One of the most common types of cancer is colorectal cancer (CRC), but in the population of patients with celiac disease is the risk of colorectal carcinoma lower than in the healthy population, as it is evidenced by recent studies [9,19,20].…”

“…It is even mentioned that CD can act as a protective condition against other cancers than lymphomas [9]. It is hypothesized that an extreme immune response elicited from damaged intestinal mucosa results in depletion of cellular components of lymph nodes manifesting as involution or cavitation and hyposplenism in CD patients [26].…”

“…Concerning lymphomas various types are described as was already mentioned above [9,16,17,27,28]. Underlying mechanisms are still poorly understood.…”