Nontropical sprue

Nontropical Sprue, also known as Celiac Disease or Gluten-Sensitive Enteropathy, is a chronic autoimmune disorder affecting the small intestine in genetically predisposed individuals. It is characterized by a reaction to gluten, a protein found in wheat, barley, and rye, leading to inflammation and villous atrophy in the small intestine. This results in impaired nutrient absorption and a wide range of clinical manifestations.

Etiology and Pathogenesis

The exact cause of Nontropical Sprue is multifactorial, involving genetic predisposition, environmental factors, and an abnormal immune response. The main genetic factors are the HLA-DQ2 and HLA-DQ8 haplotypes, which are present in the majority of patients with the disease. Environmental factors include the introduction of gluten into the diet. The immune response to gluten peptides leads to inflammation and damage to the intestinal mucosa.

Clinical Features

Symptoms of Nontropical Sprue can vary widely among individuals and may include gastrointestinal manifestations such as diarrhea, abdominal pain, and bloating, as well as systemic symptoms like anemia, osteoporosis, and neurological disorders. Some patients may present with dermatitis herpetiformis, a skin manifestation of the disease.

Diagnosis

Diagnosis of Nontropical Sprue involves a combination of serological tests for specific antibodies (such as anti-tissue transglutaminase antibodies and anti-endomysial antibodies), histological examination of a small intestine biopsy showing villous atrophy, and clinical improvement upon initiation of a gluten-free diet.

Treatment

The primary treatment for Nontropical Sprue is a strict lifelong gluten-free diet, which leads to the healing of the intestinal mucosa, resolution of symptoms, and decreased risk of complications. Patients may also require supplementation of vitamins and minerals that were deficient due to malabsorption.

Complications

Untreated or inadequately managed Nontropical Sprue can lead to various complications, including malnutrition, infertility, increased risk of certain types of cancer (such as intestinal lymphoma), and additional autoimmune disorders.

Epidemiology

Nontropical Sprue is a common disorder, particularly in populations of European descent, but it can affect individuals of any ethnic background. It is often underdiagnosed due to the wide range of symptoms and clinical presentations.