- 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 (rose bengal staining and Schirmer test) and oral (salivary flow measurement and parotid scintigraphy) tests.
- 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 may be complicated by the development of lymphoma.
- Patients with SjS present a broad spectrum of analytic 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.
Sjögren syndrome (SjS) is a systemic autoimmune disease that mainly affects the exocrine glands 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, of whom approximately 1 million have established diagnoses. The prevalence in European countries ranges between 0.60% and 3.3%. The incidence of SjS has been calculated as 4 cases per 100,000. SjS primarily affects white perimenopausal women, with a female:male ratio ranging from 14:1 to 24:1 in the largest reported 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 26–1. Although most patients present with sicca symptoms, there are various clinical and analytic features that may indicate undiagnosed SjS (Table 26–2). 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 and immunosuppressive agents play a key role in the treatment of extraglandular features.
Table 26–1. Major Clinical Manifestations of Sjögren Syndrome. |Favorite Table|Download (.pdf)
Table 26–1. Major Clinical Manifestations of Sjögren Syndrome.
|Mouth||Oral dryness (xerostomia), soreness, caries, periodontal disease, oral candidiasis, parotid swelling|
|Eyes||Ocular dryness (xerophthalmia), corneal ulcers, conjunctivitis|
|Nose, ear, and throat||Nasal dryness, chronic cough, sensorineural hearing loss|
|Skin||Cutaneous dryness, palpable purpura, Ro-associated polycyclic lesions, urticarial lesions|
|Joints||Arthralgias, nonerosive symmetric arthritis|
|Lungs||Obstructive chronic pneumopathy, bronchiectasis, interstitial pneumopathy|
|Cardiovascular||Raynaud phenomenon, pericarditis, autonomic disturbances|
|Liver||Associated hepatitis C virus infection, ...|