**6. Malignancies associated with CD**

The most important complication of CD is development of cancer. The incidence of gastrointestinal and non-gastrointestinal both is common among CD patients. The commonest among the malignancies is the gastrointestinal lymphomas accounting for 18% of all cancers. The gastrointestinal tract is the predominant site of extranodal lymphoma involvement. Primary lymphomas are less common than secondary lymphomas. Primary lymphomas typically involve any section of gastrointestinal tract from oropharynx to rectum [64]. It can involve single or multiple sites. These are usually non-Hodgkin's lymphomas although Hodgkin's lymphoma has been reported as well. The commonest site of involvement of non-Hodgkin's lymphoma is stomach, present in 68–75%, followed by small bowel than ileocecal junction. Diffuse colonic involvement is present only in 1% [65]. Distribution of primary lymphomas varies among population; gastric lymphoma is more common in United States, while intestinal lymphoma is seen in Middle East and Mediterranean areas. GI Lymphomas are commonly seen in patients with helicobacter pylori infection, autoimmune disease, immunodeficiency and immunosuppressive states, inflammatory bowel disease and CD. Both the T and B cell are commonly seen in patients with CD. The exact etiology of development of these malignancies is under controversy, both the autoimmune and inflammatory factors contribute to the risk. The T cell variant enteropathy-associated T-cell lymphoma (EATL) (**Figure 8**) involving the small intestine mostly, is the commonest malignancy seen in celiac disease, although uncommonly seen [66, 67]. It is most commonly found in adult males with a median age of 60 years. EATL patients mostly presented with acute bleeding, perforation or obstruction, or it should always be suspected if there is clinical deterioration of CD, despite on a strict gluten free diet. It is highly suggested to screen a patient for CD, even if not diagnosed before, if

presented with EATL. Patients with enteropathy associated T cell lymphoma of the small intestine, involving jejunum demonstrates large circumferential ulcer without overt mass. The involved area typically shows lymphoma, while the non-involved sites usually show villous atrophy.

The other variant of EATL, ulcerative enteritis, is another complication of long standing and refractory sprue. It presents with abdominal pain, nausea, vomiting and diarrhea. The other complication of CD includes intestinal ulceration independent of lymphoma and so called refractory sprue and collagenous sprue (**Figure 9**). It is a clinicopathological entity characterized by diarrhea and malabsorption accompanied by the histological findings of subepithelial collagen deposition and severe villous atrophy of small bowel mucosa [68]. The occurrence of collagenous sprue has been seen in patients with celiac disease, tropical sprue, milk intolerance and common variable immunodeficiency states [69]. Regardless of etiology it has a poor prognosis. Collagenous sprue associated with CD usually does not respond to gluten free diet and has a poor prognosis [70].

**Figure 8.** *Lymphoma.*

**79**

*Complications of Celiac Disease*

ment of malignancies in CD.

**7. Hyposplenism in CD**

**9. Kidney disease in CD**

**11. Dental defects in CD**

mended [71, 72].

*DOI: http://dx.doi.org/10.5772/intechopen.80465*

**8. Venous thromboembolism in CD**

tion with other disease is relatively high.

**10. Idiopathic pulmonary hemosiderosis in CD**

The risk of other digestive tract malignancies is also commonly seen in CD patients, including oropharyngeal, colorectal, small intestinal adenocarcinoma and hepatocellular carcinomas. In oropharyngeal carcinomas, the commonest is the squamous cell carcinoma. In contrast to gastrointestinal carcinomas, the nongastrointestinal malignancies, including breast carcinomas are not seen commonly in CD patients. There is no evidence that gluten free diet may decrease the develop-

One of the earliest manifestations of CD is hyposplenism, though the exact mechanism of development of hyposplenism is not known. Due to hyposplenism there is increased susceptibility of infection due to encapsulated bacteria especially. Vaccination against prophylaxis for pneumococcus is not highly recom-

Hypercoagulability with elevated homocysteine level and low vitamin-K-dependent anti-coagulant proteins (protein C & S) are relatively common in CD patients. Due to these factors, i.e. increased homocysteine level and decreased protein C &S, along with autoimmunity of CD, there might be increased susceptibility to venous

Glomerular IgA deposition is common, occurring in as many as one-third of patients. Although the clinical manifestation is not evident, due to no associated complement deficiency. This indicates that a high circulating load of polyclonal IgA is not adequate to cause nephritis, but other abnormalities of IgA are necessary to translate into mesangial activation and glomerular injury. This mesangial IgA deposition is also seen in healthy individuals from 3 to 16% [74]. Thus its associa-

Lane-Hamilton syndrome, the co-existence of CD and idiopathic pulmonary hemosiderosis, is not an uncommon entity in CD patients, although the exact prevalence is not known [75]. It is a rare lung disease characterized by alveolar capillary bleeding and accumulation of hemosiderin in the lungs. Diffuse alveolar hemorrhage is characterized by hemoptysis, dyspnea, alveolar opacities on chest X-ray and anemia. It may lead to iron loss through swallowing of iron-laden alveolar

Oral manifestations are overlooked in CD patients; the long list of clinical signs and symptoms associated with CD includes dental enamel hypoplasia, aphthous

or bronchial epithelial cells. This may lead to functional iron deficiency [76].

thromboembolism. Although not commonly observed in CD patients [73].

**Figure 9.** *Collagenous colitis.*

#### *Complications of Celiac Disease DOI: http://dx.doi.org/10.5772/intechopen.80465*

The risk of other digestive tract malignancies is also commonly seen in CD patients, including oropharyngeal, colorectal, small intestinal adenocarcinoma and hepatocellular carcinomas. In oropharyngeal carcinomas, the commonest is the squamous cell carcinoma. In contrast to gastrointestinal carcinomas, the nongastrointestinal malignancies, including breast carcinomas are not seen commonly in CD patients. There is no evidence that gluten free diet may decrease the development of malignancies in CD.
