Nonresponsive Celiac Disease

Whilst most patients with celiac disease respond appropriately to a gluten-free diet, usually with responses to symptoms occurring within days to weeks of institution of the diet, a small proportion of patients (approximately 5%) do not have the expected complete response to a gluten-free diet or they have a relapse of symptoms while apparently on a gluten-free diet. This scenario termed 'nonre-sponsive celiac disease' is multifactorial in nature.

The single most common cause of continued or relapsing symptoms in patients with celiac disease is that of inadvertent gluten ingestion. There are many ways in which gluten can get into the diet, and in one series the most common source was commercial cereal in which minor ingredients were derived from the offending grains. However, other sources such as communion wafers and environmental contamination with flour, particularly of baked goods, are also possible.

In patients whose serologic tests have returned to normal and where a careful dietary review, including a detailed food record, does not reveal any potential source of gluten contamination, the occurrence of a second associated disease or a complication of celiac disease must be considered. A common associated disorder would be microscopic colitis, either lymphocytic or collagenous. Typically, these patients will have watery diarrhea whereas symptoms related to malabsorption such as weight loss, bloating, and steatorrhea will have resolved. The patient will continue to have watery diarrhea or may, indeed, develop watery diarrhea while on a gluten-free diet. The taking of biopsies from the colon can readily identify this condition. Whilst in some patients adhering to a strict gluten-free diet may improve the colitis, in many circumstances, it does not or it has not sufficed. The use of empiric therapy such as Pepto-BismolĀ®, loperamide, or, in some circumstances, delayed release budesonide may be valuable. Another cause of continued diarrhea is disaccharidase deficiency such as lactose intolerance. In most patients with celiac disease, the lactose intolerance that occurs is secondary to the injury and resolves its treatment. In a few unfortunate patients there may be a genetic predisposition to lactose intolerance. Avoidance of lactose or the use of lactase enzyme supplementation may suffice for correction of symptoms. In patients who have continued steatorrhea but in whom small bowel biopsies are found to have become normal, pancreatic exocrine insufficiency or bacterial overgrowth syndrome might be considered.

Where the small intestine has failed to recover histologically, particularly in patients who have continued symptoms and signs of malabsorption, the diagnosis of refractory sprue is made. This condition is often associated with severe illness, significant bone disease, and hypoalbuminemia. These patients are particularly prone to ulceration in the proximal small intestine, so-called ulcerative jejunitis. Some have clonal expansion of T cells within their intestine. These patients are probably entering a pre-lymphoma state and the mortality in these circumstances is high with a relatively poor response to immunosuppres-sion; many will progress to lymphoma within 5 years. Other patients appear to have refractory sprue but without clonality, and they tend to respond much better to immunosuppression. This probably represents a now self-perpetuating autoimmune process within the intestine. The rare case of collagenous sprue, which has features similar to celiac disease but is characterized by a thick layer of collagen in the intestine subepithelial layer in the colon, typically responds poorly to all therapies and often require long-term nutritional support.

The approach to diagnosing and treating nonre-sponsive celiac disease is outlined in Figure 3.

Figure 3 Flow chart for diagnosing and treating celiac patients. The proper procedure for diagnosing a patient who potentially has celiac disease, education and treatment of that patient, followed by the steps that need to be taken in the event that the patient is not responsive to a gluten-free diet are illustrated in a flow chart. GC, gluten challenge.

Figure 3 Flow chart for diagnosing and treating celiac patients. The proper procedure for diagnosing a patient who potentially has celiac disease, education and treatment of that patient, followed by the steps that need to be taken in the event that the patient is not responsive to a gluten-free diet are illustrated in a flow chart. GC, gluten challenge.

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