232 M. Ramos-Casals et al.

Table 16.2 Previous reported cases of biopsy-proven vasculitis involving the gastrointestinal tract in patients with primary Sjögren syndrome [41]

These latter studies conflict with the notion that patients with primary SS should be evaluated routinely for celiac disease [40].

16.6Intestinal Vasculitis

Among patients with primary SS, systemic vasculitis only rarely involves the gastrointestinal tract. Of the 19 reports of SS patients with systemic necrotizing vasculitis [41], 13 had gastrointestinal tract involvement (Table 16.2). In addition, of the 19 reported deaths of SS patients due to vasculitis, the two main causes were CNS involvement in 6 and gastrointestinal perforation in 5. Cryoglobulins were determined in 12 of these patients and were positive in 10 (83%) cases. In spite of its rarity, gastrointestinal vasculitis, often related to cryoglobulinemia, should be considered as a life-threatening situation in patients with primary SS.

16.7Other Intestinal Diseases

Recent studies have described other intestinal diseases in patients with primary SS. Lidén et al. [42] found an inflammatory mucosal response after rectal exposure to cow’s milk protein in 38% of patients with primary SS. The same authors also found [43] a similar reaction after gluten exposure in 20% of patients. However, the two studies found no correlation between sensitivity to gluten or cow’s milk protein with food intolerance or gastrointestinal symptoms.

16.8Conclusion

A number of studies have analyzed the prevalence and clinical significance of gastrointestinal diseases in patients with primary SS, including esophageal dysfunction, gastritis, H. pylori infection, and intestinal malabsorption, with differing results. Esophageal and gastric diseases are frequent in primary SS, but it is not clear whether they are etiopathogenically related to SS. In contrast, intestinal involvement should be considered as one of the less-frequent extraglandular manifestations of primary SS, with isolated cases of malabsorption or vasculitis being reported. Some issues remain unresolved, including the prevalence of celiac disease in patients with primary SS and the possible role of hypersensitivity to cow’s milk protein in irritable bowel syndrome, which is often presented by patients with primary SS. However, current knowledge suggests that the majority of gastrointestinal diseases diagnosed in patients with primary SS have a similar prevalence to that found in nonautoimmune patients with a similar epidemiological profile.

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Chapter 17

Liver Involvement in Sjögren’s Syndrome

George E. Fragoulis, Fotini N. Skopouli, Carlo Selmi, and M. Eric Gershwin

Contents

G.E. Fragoulis (*)

Department of Pathophysiology, School of Medicine,

National University of Athens, Athens, Greece

F.N. Skopouli

Department of Dietetics and Nutritional Science,

Harokopio University of Athens, Athens, Greece

C. Selmi

Division of Rheumatology, Allergy and Clinical Immunology,

University of California at Davis, Davis, CA, USA

Department of Translational Medicine, IRCCS Istituto Clinico Humanitas, University of Milan, Rozzano (MI), Italy

M.E. Gershwin

Division of Rheumatology, Allergy and Clinical Immunology,

Genome and Biomedical Sciences Facility, University of California at Davis, Davis, CA, USA