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What is Sjogren's syndrome?
a slowly progressive autoimmune inflammatory disorder associated with lymphoproliferation and mononuclear cell infiltration of the exocrine glands. This condition has a 9:1 female predominance and usually affects patients in midlife. Sjögren syndrome may occur as a primary disorder but commonly presents secondary to another autoimmune disease. This condition most often develops in association with rheumatoid arthritis and systemic lupus erythematosus but also occurs in patients with systemic sclerosis and inflammatory myopathy.
What are the clinical manifestations of Sjögren syndrome?
- keratoconjunctivitis sicca, which causes xerophthalmia (dry eyes), and xerostomia (dry mouth)
- Vaginal dryness and parotid gland enlargement are frequently present, and fatigue and arthralgia are common.
- inflammatory polyarthritis.
- Additional systemic features of Sjögren syndrome include cutaneous vasculitis, peripheral neuropathy, vasculitis that may be associated with mononeuritis multiplex, and interstitial nephritis with associated distal renal tubular acidosis.
- Pulmonary involvement may develop in patients with Sjögren syndrome and most commonly manifests with interstitial lung disease; however, bronchial and bronchiolar disease also may occur.
How is Sjögren syndrome diagnosed?
- Abnormal findings on the Schirmer test, which measures moisture under the lower eyelids, are consistent with Sjögren syndrome. Referral to an ophthalmologist for rose bengal staining also may be warranted. In this study, visualization of small conjunctival and corneal punctate lesions caused by microtrauma associated with ocular dryness suggests a diagnosis of Sjögren syndrome.
- The presence of xerophthalmia and xerostomia accompanied by anti-Ro/SSA and anti-La/SSB antibody positivity and abnormal findings on the Schirmer test or rose bengal staining have 94% sensitivity and specificity for primary Sjögren syndrome. Pathologic diagnosis of this condition can be confirmed if biopsy specimens of a labial salivary gland reveal focal lymphocytic infiltration.