Celiac sprue (review)

Celiac sprue may be defined as a model autoimmune disease with known trigger (gluten), a tight genetic linkage (with HLA-DQ2 and HLA-DQ8) and a specific humoral autoimmune response (autoantibodies to tissue transglutaminase, tTG). Gliadin peptides are repeatedly presented to HLA-DQ2 and HLA-DQ8 positive cells and induce an immune response in small-intestinal mucosa. tTG is a specific endomysial autoantigen released during cellular stress and by deamidation of gliadin peptides as well as by binding with them facilitates their interaction with HLA-DQ2 and HLA-DQ8 cells. CS is the consequence of an inappropriate by T-cells mediated immune reaction to gluten. The diagnosis is based on criteria of the European Society of Paediatric Gastroenterology, Hepatology and Nutrition revised in 1990. The availability of sensitive and specific detection methods of serum antibodies to endomysium (AEA) and tTG (AtTGA) was followed by recognition of a broad spectrum of both the clinical presentation and histologic changes of intestinal mucosa in CS. The majority of patients have atypical clinical symptoms. The present prevalence of CS amounts to approximately 1:250. The following forms of CS are distinguished: classic (typical), latent, potential, subclinical, and silent. CS is frequently associated with other diseases and many of them are also of autoimmune origin. Serologic testing is indicated in subjects with atypical symptoms and autoimmune diseases. In cases with distinct suspicion biopsy should be always performed irrespective of the serologic tests. In refractory sprue the cryptic form of enteritis associated T-cell lymphoma should be excluded. Gluten-free diet is the cornerstone of CS therapy. The Alimentary Codex in individual countries admits different amounts of residual gluten in gluten-free products. In future years new basic knowledge as well as practical diagnostic and therapeutic recommendations may be expected.     

Celiac sprue

Celiac sprue, celiac disease, or gluten-sensitive enteropathy, is a malabsorption disorder of the small intestine that occurs after ingestion of wheat gluten in genetically susceptible individuals. This disease is characterized by intestinal malabsorption associated with villous atrophy of the small intestinal mucosa, clinical and histological improvement after adherence to strict gluten free diet, and relapse when gluten is reintroduced. Celiac sprue has a high prevalence in Western Europe and North America where it is estimated to affect 1:120 to 1:300 individuals. The pathogenesis of celiac sprue is related to inappropriate intestinal T-cell activation in HLA-DQ2 positive individuals triggered by antigenic peptides from wheat gluten or prolamins from barley and rye. Although previously thought to be mainly a disease of childhood onset, the diagnosis is increasingly being made in adults. There are a wide variety of presentations, which range from asymptomatic forms to severe diarrhea, weight loss and nutritional deficiencies. Extraintestinal manifestations including anemia, osteopenia or neurological disorders and associated conditions such as diabetes or hypothyroidism are commonly present. The availability of highly sensitive and specific serologic markers has dramatically facilitated the diagnosis of celiac sprue. However, the demonstration of characteristic histological abnormalities in a biopsy specimen of the small intestine remains the mainstay of diagnosis. Treatment consists of life-long avoidance of dietary gluten to control symptoms and to prevent both immediate and long-term complications.     

Celiac disease (celiac sprue, gluten-sensitive enteropathy)

In spite of the remarkable progress made in the last years, the perspectives in coeliac disease is still an enigma that has maintained this clinical entity a subject open to investigation. However, particular attention is deserved to the recent contributions related to the role of the histocompatibility antigens (HLA system), which is basically controlled by inherited transmission but can behave, in certain cases as gluten-sensitive "receptors" at the level of brush-border membrane. The coupling of these receptors with antigenic fractions of gluten in a sensitive person should be a starting point for a sequence of immunological events, with its several intestinal and general implications.     

Celiac sprue and related diseases

Opinion statement  

Celiac sprue: another autoimmune syndrome associated with hepatitis C

OBJECTIVE: Celiac sprue is being diagnosed with increasing frequency by screening individuals with epidemiologically associated autoimmune syndromes. We sought to test our hypothesis that hepatitis C also may predispose to celiac sprue because it can trigger autoimmune reactions. METHODS: Two hundred fifty-nine consecutively evaluated patients with chronic hepatitis C infection, 59 with autoimmune liver disease, 137 with other hepatic diseases, 356 with various GI syndromes, and 221 normal volunteers underwent serologic screening for celiac sprue. Patients with antigliadin, antiendomysial, and antitissue transglutaminase antibodies in serum underwent duodenoscopy and biopsy. RESULTS: There was a statistically significantly higher prevalence of antigliadin antibody in all groups of patients with liver disease compared with GI controls and normal controls. However, only patients with hepatitis C (n = 3; 1.2%) or autoimmune liver disease (n = 2; 3.4%) had antiendomysial/antitissue transglutaminase antibody in serum. One of 221 normal volunteers (0.4%) was antigliadin, antiendomysial, and antitissue transglutaminase positive; this individual also was found to have hepatitis C (previously undiagnosed). Each of these six individuals had mild intestinal symptoms, duodenal histopathology consistent with celiac sprue, and the celiac-associated HLA-DQ2 allele. Five of the six followed a prescribed gluten-free diet and experienced symptomatic improvement. CONCLUSION: Celiac sprue is epidemiologically associated with chronic hepatitis C infection and with autoimmune liver disease. Because hepatitis C is much more frequently encountered than autoimmune liver disease, hepatitis C appears to be the most common hepatic disease associated with the development of celiac sprue.     

Celiac sprue complicated by lymphoma presenting with multiple gastric ulcers

A 40-yr-old woman with celiac sprue, which had responded clinically and histologically to gluten elimination, subsequently developed gastrointestinal lymphoma. Although this has been described in the small intestine of patients with celiac sprue, the unique feature in this patient was her initial presentation with multiple gastric ulcers refractory to conventional medical therapy. This case demonstrates that lymphoma complicating celiac sprue may present with multiple refractory gastric ulcers in addition to those occurring in the small intestine.     

Celiac sprue and ulcerative colitis in three South Asian women.

The association of celiac disease and inflammatory bowel disease has not been described in females of South Asian descent. We report three women, aged 33, 43 and 46 years, of South Asian origin, with both ulcerative colitis and celiac disease.     

Association of lymphocytic (microscopic) colitis with tropical sprue

Abstract Lymphocytic (microscopic) colitis is a disease of unknown aetiology which manifests as long-standing intermittent diarrhoea. Diagnosis is confirmed on histological examination of the colon. An association of this uncommon disease with tropical sprue is described. Tetracycline therapy resulted in a favourable clinical response.