We searched MEDLINE (1977–2004) with the search terms “Sjögren's”, “Sjogren”, “keratoconjunctivitis sicca”, “xerostomia”, “salivary gland”, “lacrimal gland”, and “systemic lupus”, “systemic sclerosis” in association with ocular or oral manifestations. Several reviews and books were cited because they provide comprehensive overviews that are beyond the scope of this Seminar. The reference list was subsequently modified during the peer-review process on the basis of comments from the
SeminarSjögren's syndrome
Section snippets
Diagnosis: criteria and pitfalls
There is little disagreement among rheumatologists about the diagnosis of Sjögren's syndrome in a patient with obvious findings on physical examination of keratoconjunctivitis sicca, dry mouth, and the presence of ANA and antibodies to SS-A or SS-B. In these patients, the important issue is the extent of extraglandular disease and type of therapy needed.
In patients with milder sicca symptoms and less characteristic antibody profiles, diagnosis and therapy can be difficult. Although there is
Pathogenesis and relation to symptoms
Despite extensive study of the underlying cause of Sjögren's syndrome, the pathogenesis remains obscure. In broad terms, the pathogenesis is multifactorial; environmental factors are thought to trigger inflammation in individuals with a genetic predisposition to the disorder. In contrast to many other organ-specific autoimmune disorders, affected tissue can be obtained easily in Sjögren's syndrome by minor-salivary-gland biopsy; thus, researchers have an opportunity to study interaction between
Clinical features of the eyes
The characteristic ophthalmological finding in Sjögren's syndrome is keratoconjunctivitis sicca. In assessment of a patient complaining of dry eyes, the important aim is to find out whether the objective signs of dry eyes are commensurate with his or her symptoms. Methods to measure the integrity of the corneal surface and tear film include staining with Rose-Bengal, fluorescein, and lissamine green dye and the tear break-up time. A rheumatologist can rapidly and inexpensively assess the ocular
Oral symptoms and signs
Dryness of the mouth makes swallowing of food and even talking difficult, owing to dryness of the buccal mucosa. However, a dry mouth is not necessarily painful. The sudden development of pain in the mouth should stimulate a search for signs of angular cheilitis or erythematous petechial-type lesions on the palate (commonly under dentures); such findings suggest oral candidosis.45 Physicians46 generally do not appreciate the contribution of oral symptoms to quality of life. A series of tools
Extraglandular systemic manifestations
Systemic manifestations (table) are subdivided into non-visceral (skin, arthralgia, myalgia) and visceral (lung, heart, kidney, gastrointestinal, endocrine, central and peripheral nervous system). There is a close overlap in symptoms and signs between systemic lupus erythematosus and Sjögren's syndrome.9 We discuss the disorders more common in Sjögren's syndrome.
Skin manifestations of small vessels include palpable and non-palpable purpura in association with cryoglobulinaemia and
Pharmacological management
Non-visceral manifestations such as arthralgia and myalgia are generally treated with salicylates, non-steroidal agents, and hydroxychloroquine. As in systemic lupus erythematosus, corticosteroids are effective but limited by their usual side-effects including osteoporosis, diabetes, cardiovascular effects, and mood disruption. Patients with Sjögren's syndrome have greater problems with corticosteroids, including acceleration of periodontal disease and oral candidosis. Another difficulty is low
Search strategy and selection criteria
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