Sjögren’s Syndrome: Beyond Sicca Involvement

Abstract

Sjogren syndrome (SS) is a systemic autoimmune disease that presents with sicca symptomatology of the major mucosal surfaces. The clinical spectrum of this condition often extends to systemic involvement (extraglandular manifestations) and may be complicated by the development of lymphoma. SS is one of the most prevalent autoimmune diseases (with an estimated 0.5–3 million sufferers in the United States), and primarily affects perimenopausal women. When sicca symptoms appear in a previously healthy ­person, the syndrome is classified as primary SS. This chapter summarizes recent work focused on extending and characterizing the acute and/or complex clinical presentations of patients with primary SS, including a wide variety of symptoms and the involvement of internal organs. As a general rule, the management of extraglandular features in primary SS should be targeted to the specific organ involved. The mainstays of such treatment regimens remain glucocorticoids and immunosuppressive agents. Such therapies are more likely to affect favorably the extraglandular manifestations of SS, even though the patients’ most intense complaints often pertain to sicca features. Severe, life-threatening involvement has rarely been reported in primary SS. There are now substantially more data on the outcome of patients with primary SS, which indicate that patients with a predominantly extraepithelial expression (often associated with cryoglobulinemia) should be monitored and managed differently from patients with a predominantly periepithelial or sicca-limited disease.