Celiac disease: From pathophysiology to treatment

Abstract

Celiac disease, also known as "celiac sprue", is a chronic inflammatory disorder of the small intestine, produced by the ingestion of dietary gluten products in susceptible people. It is a multifactorial disease, including genetic and environmental factors. Environmental trigger is represented by gluten while the genetic predisposition has been identified in the major histocompatibility complex region. Celiac disease is not a rare disorder like previously thought, with a global prevalence around 1%. The reason of its under-recognition is mainly referable to the fact that about half of affected people do not have the classic gastrointestinal symptoms, but they present nonspecific manifestations of nutritional deficiency or have no symptoms at all. Here we review the most recent data concerning epidemiology, pathogenesis, clinical presentation, available diagnostic tests and therapeutic management of celiac disease.

Keywords: Celiac disease; Diagnosis; Epidemiology; Pathogenesis; Treatment.

Figure 1

The “iceberg model” idealizing the interplay between celiac disease genetic makeup and exposure to gluten, the environmental trigger of the disease.

Figure 2

Histological features of celiac disease. A: Example of tissue scored as Marsh 2 characterized by lymphocytic enteritis with crypt hyperplasia: Intraepithelial lymphocytosis and elongation and branching of crypts in which there is an increased proliferation of epithelial cells; B: Example of tissue scored as Marsh 3A characterized by partial villous atrophy, the villi are blunt and shortened. Arbitrarily, samples are classified as partial villous atrophy if the villus-crypt ratio was less than 1:1 (Objective magnification × 4, inset × 10).