32.6.3Association of SS with Coeliac Disease

Several studies have found an increased incidence of coeliac disease in pSS. Fifteen percent of 34 pSS patients investigated prospectively had coeliac disease [72]. 4.5% of 111 Hungarian patients with pSS had coeliac disease, compared to 0.5% in the general population [73]. A UK cohort had a lower prevalence of coeliac disease at 0.9% [2]. Three of 141 pSS patients had coeliac disease in a Mexican study [74]. Luft et al. found that 6 of 50 (12%) patients with pSS had antibodies to tissue transglutaminase (TTG), with 5 of the 6 having histological evidence of coeliac disease [74]. This contrasts with a study by Roth et al. who found that only 2 of 77 patients with pSS had IgA antibodies to transglutaminase, and none had IgG antibodies [75]. A study of 334 patients with coeliac disease found that 3.3% of patients had SS, compared to 0.3% of controls [76].

32.7Conclusions

The key messages to emerge from this chapter include:

1. Although SS is principally a disease of the exocrine glands, it may can a variety of tissues beyond the exocrine glands and lead to significant cumulative co-morbidity. A wide range of clinical features have been linked to SS, and other autoimmune diseases frequently co-exist with this condition. Autoimmune hypothyroidism is the organ-specific autoimmune disease that is most commonly associated with SS, followed by autoimmune liver disease. Thus, making a diagnosis of SS is not the ‘end of the story’. Life-long follow-up is required, with vigilance for the development of extra-glandular manifestations, other autoimmune diseases, and lymphoma. In particular, anti-thyroid antibodies should be checked at diagnosis, and thyroid function measured regularly thereafter.

2. The terminology of ‘secondary’, ‘associated’ and ‘overlap’ SS needs clarification. 3. SS secondary to RA is milder than the primary form of the disease, with different genetic associations. SS accompanying SLE (SLE-SS) and primary SS are clinically, serologically, and genetically similar. Patients with SLE-SS tend to be older and have milder lupus than patients with SLE alone. Sicca symptoms are frequent in SSc, but are often due to pure glandular fibrosis. SS accompanying SLE and SSc may be better described as ‘associated’ than ‘secondary’. By contrast,

in RA ‘secondary SS’ is an accurate description.

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Chapter 33

Cancer

Elke Theander and Eva Baecklund

Contents

E. Theander (*)

Department of Rheumatology, Skåne University Hospital,

Malmö, Sweden

E. Baecklund

Rheumatology Unit, Department of Medical Sciences, Uppsala University,

Uppsala, Sweden