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ESSENTIALS OF DIAGNOSIS

  • Sjögren syndrome (SjS) is a systemic autoimmune disease that presents with sicca symptomatology of mucosal surfaces.

  • The main sicca features (xerophthalmia and xerostomia) are determined by specific ocular tests (corneal stainings and Schirmer test) and oral investigations (salivary flow measurement and parotid scintigraphy).

  • The histologic hallmark is a focal lymphocytic infiltration of the exocrine glands, determined by a biopsy of the minor labial salivary glands.

  • The spectrum of the disease includes systemic features (extraglandular manifestations) in some patients, and is complicated by the development of lymphoma in a small percentage of patients.

  • Patients with SjS can be characterized by a broad spectrum of laboratory features (cytopenias, hypergammaglobulinemia, and high erythrocyte sedimentation rate) and autoantibodies, of which antinuclear antibodies are the most frequently detected, anti-Ro/SS-A the most specific, and cryoglobulins and hypocomplementemia the main prognostic markers.

General Considerations

Sjögren syndrome (SjS) is a systemic autoimmune disease that affects the exocrine glands prominently and usually presents as persistent dryness of the mouth and eyes due to functional impairment of the salivary and lacrimal glands. An estimated 2–4 million persons in the United States have SjS, the majority of whom have this condition on a secondary basis—secondary to another underlying rheumatologic disorder. The prevalence of SjS in European countries is estimated to range between 0.05% and 0.72% of the general population, and the annual incidence of primary SjS has been calculated to be on the order of 3–10 new cases per 100,000/year. SjS primarily affects white perimenopausal women, with a female:male ratio of 14:1 in the largest reported international series. The disease may occur at all ages but typically has its onset in the fourth to sixth decades of life. When sicca symptoms appear in a previously healthy person, the syndrome is classified as primary SjS. When sicca features are found in association with another systemic autoimmune disease, most commonly rheumatoid arthritis, systemic sclerosis, or systemic lupus erythematosus, it is classified as associated SjS.

Major clinical manifestations are summarized in Table 24–1. Although most patients present with sicca symptoms, there are various clinical and laboratory features that may indicate undiagnosed SjS (Table 24–2). The variability in the presentation of SjS may partially explain delays in diagnosis of up to 10 years from the onset of symptoms. SjS is a disease that can be expressed in many guises depending on the specific epidemiologic, clinical, or immunologic features. The management of SjS is centered mainly on the control of sicca features, using substitutive and oral muscarinic agents. Glucocorticoids, immunosuppressive agents, and biologics play a key role in the treatment of systemic disease.

Table 24–1.Major clinical manifestations of Sjögren syndrome.

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