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70 CPD ACTIVITY COELIAC DISEASE COELIAC DISEASE Morna Falkland Morna Falkland is a medicines information pharmacist who has worked in the hospital sphere in Canberra. LEARNING OBJECTIVES After completing this CPD activity, pharmacists should be able to: • Describe the underlying causality of the development of coeliac disease. • List the symptoms and complications of uncontrolled coeliac disease. • Outline the approach to management of coeliac disease. 2016 Competency Standards: 3.1, 3.2, 3.6. CPD Accreditation Number: A2007RP2 (exp: 30/06/2022) Coeliac disease is an autoimmune disease caused by intolerance to gluten, a protein found in oats, wheat, barley, rye and related grains. In genetically predisposed individuals the immune reactions result in inflammation and damage to the lining of the small intestine, interference with absorption of nutrients from food, and subsequent long-term complications. Coeliac disease affects at least one per cent of the Australian population.1 Pathophysiology Nearly all people with coeliac disease carry one of two susceptibility genes: human leukocyte antigen (HLA)-DQ2 and HLA-DQ8 which are seen in 99 per cent of patients with the condition.2 In the submucosa, gluten peptides are de-amidated to glutamate by tissue transglutaminase (tTG), an enzyme normally involved in collagen cross-linking and tissue remodelling.2 The negatively charged glutamate side chains have a greater potential to be recognised as immunogenic. These gluten-derived antigens are then recognised by the HLA-DQ2 and HLA-DQ8 found on antigen-presenting cells, leading to activation of CD4+ helper T (Th) cells. Stimulation of Th cells causes intestinal inflammation resulting in cell death and tissue remodelling, with villous atrophy and crypt hyperplasia.3 High-risk groups The presence of coeliac disease is Small intestine with normal villi, and villous atrophy. Diagram shows changes in intestinal. coeliac disease manifested by blunting of villi. RETAIL PHARMACY • JUL 2020 three to 10-fold higher in the following patient groups:1 • First and second-degree relatives of patients with coeliac disease. • Down syndrome. • Turner syndrome. • Selective IgA deficiency. • Other conditions that have autoimmune features such as type 1 diabetes, thyroid disease and liver disease. A positive family history of coeliac disease carries the strongest predictive value for the disease. The risk of coeliac disease in people who have an affected family member is 10 per cent.1 While coeliac disease can develop at any age, the median age at diagnosis is 40 years.2 Symptoms Symptoms of coeliac disease can vary and may be severe in some people, while others are asymptomatic with no obvious symptoms. In the past, coeliac disease usually presented in infants and young children six to 24 months of age with chronic diarrhoea, abdominal distension, malabsorption and failure to thrive. Now coeliac disease tends to present later, with milder gastrointestinal or non- gastrointestinal symptoms. Symptoms can include any of the following:5 • Gastrointestinal symptoms, eg, diarrhoea, constipation, nausea, vomiting, flatulence, cramping, bloating, abdominal pain, steatorrhoea. • Prolonged fatigue, weakness, lethargy. • Iron deficiency anaemia and/or other vitamin and mineral deficiencies. • Osteoporosis. • Failure to thrive, irritability or delayed growth and development in children. • Unexpected weight loss. • Bone and joint pains. • Recurrent mouth ulcers and/or swelling of mouth or tongue. • Altered mental alertness and irritability. • Skin rashes such as dermatitis herpetiformis. Atypical coeliac disease lacks the typical gastrointestinal symptoms and presents 2 CPD CREDITS E S A E S I D C O E L I A C