What is the primary management strategy for celiac disease?

Primary Management of Celiac Disease

The primary management strategy for celiac disease is a strict, lifelong gluten-free diet (GFD), which is the only effective and safe treatment currently available. 1, 2

Implementation of the Gluten-Free Diet

• A GFD requires complete elimination of all products containing wheat, barley, and rye proteins 2
• Patients should be referred to a dietitian for nutritional assessment and counseling about implementing a GFD 2
• The goal of treatment is to relieve symptoms, achieve mucosal healing, avoid complications, and maintain good quality of life with a nutritionally complete GFD 1
• Pure oats may be safe for most patients but should be introduced slowly with monitoring for adverse reactions 2
• Alternative gluten-free grains such as buckwheat, amaranth, quinoa, millet, and sorghum should be incorporated to improve the nutritional profile of the diet 2

Nutritional Considerations

• Testing for micronutrient deficiencies including iron, folate, vitamin D, and vitamin B12 is recommended at diagnosis 2
• Treatment of identified nutritional deficiency states is essential 1, 2
• Bone mineral density assessment is recommended to evaluate for osteoporosis 1, 2
• The GFD should be balanced to avoid nutrient deficiencies while also preventing excess weight gain, which has become increasingly common in celiac patients 3
• Focus should be placed on natural gluten-free foods including extra-virgin olive oil, milk, yogurt, plant foods, and fish rich in omega-3 fatty acids 2

Follow-up and Monitoring

• Regular follow-up with a healthcare team including a physician and dietitian is necessary 1, 2
• Monitoring adherence to GFD can be done through a combination of clinical assessment, serologic testing, and dietary review 2
• In adults, intestinal mucosa heals more slowly and less completely than in children, so serologic normalization doesn't necessarily indicate complete mucosal healing 1
• Follow-up biopsies may be considered but are not mandatory if the patient is asymptomatic on a GFD 1
• Follow-up biopsies should be undertaken in patients whose condition does not respond to a GFD 1

Management of Non-responsive Celiac Disease

• For patients with persistent or recurrent symptoms despite a GFD, a careful review of dietary history for inadvertent gluten ingestion should be conducted 1
• If gluten ingestion is not suggested by dietary review or positive serology, other conditions should be considered, including: 1
  • Microscopic colitis
  • Pancreatic exocrine insufficiency
  • Bacterial overgrowth
  • Disaccharidase deficiency
• Refractory sprue is a rare entity with high morbidity and mortality, defined as continued malabsorption and villous atrophy despite adherence to a strict GFD 1

Benefits of Gluten-Free Diet

• Compliance with a GFD is protective against the development of non-Hodgkin's lymphoma 1
• Treatment with a GFD results in substantial improvement in nutritional parameters 1
• Improvements in bone mineral density occur with the greatest gains in the first years of the GFD 1
• For patients with dermatitis herpetiformis (a skin manifestation of celiac disease), more than 70% are able to slowly wean off dapsone over 24 months when following a strict GFD 1

Challenges and Future Directions

• Adherence to a strict GFD can be challenging due to concerns about social isolation, risk of gluten contamination, high cost, and poor quality and taste of gluten-free products 4
• While alternative therapies are being developed, they are intended to complement rather than replace the GFD 5, 6
• Current therapeutic approaches in development target different aspects of celiac disease pathophysiology, but none have yet completed phase III clinical trials 6
• The most promising supplemental drugs currently in development are ALV003 and ZED-1227 6