Primary Sjögren's Syndrome and Celiac Disease: Causal Association or Serendipity?

Primary Sjögren's syndrome (pSS) is an autoimmune disease associated with other autoimmune diseases like thymoma, hypothyroidism or mucosa-associated lymphoid tissue lymphomas. Celiac disease (CD) is a gluten sensitive enteropathy that causes small bowel mucosal damage in genetically susceptible individuals. Several immunologic alterations and immune-associated diseases, for example, insulin dependent diabetes mellitus, autoimmune thyroid disease, and connective tissue disorders, have been described in CD. We report 3 of 141 pSS patients who also had CD and review literature that suggests that CD is increased in patients with pSS. Successful treatment of CD did not relieve sicca symptoms or signs; thus, the two diseases must be treated independently. Although intestinal inflammation can occur in patients with pSS, diarrhea should be evaluated for CD and other causes. Because of the occasional severity of CD, clinicians must be aware of the possible association with pSS.