An immunologic disorder characterized by progressive lymphocytic destruction of exocrine glands most frequently resulting in symptomatic eye and mouth dryness; can be associated with extraglandular manifestations; predominantly affects middle-age females; may be primary or secondary when it occurs in association with other autoimmune diseases. Lymphoma can occur in 6% with glandular MALT being most common.
- Constitutional: fatigue
- Sicca symptoms: KCS and xerostomia
- Dryness of other surfaces: nose, vagina, trachea, skin
- Extraglandular features: arthralgia/arthritis, Raynaud's, lymphadenopathy, interstitial pneumonitis, vasculitis (usually cutaneous), nephritis, lymphoma
- History and physical examination: with special attention to oral, ocular, lymphatic examination and presence of other autoimmune disorders.
- Presence of autoantibodies is a hallmark of disease (ANA, RF, anti-Ro/SS-A, anti-La/SS-B).
- Other laboratory tests: ESR; CBC; renal, liver, and thyroid function tests; serum protein electrophoresis (SPEP) (hypergammaglobulinemia or monoclonal gammopathy common); UA.
- Ocular studies: to diagnose and quantitate KCS; Schirmer's test, Rose Bengal staining.
- Oral examination: unstimulated salivary flow, dental examination.
- Labial salivary gland biopsy: demonstrates lymphocytic infiltration and destruction of glandular tissue.
International classification criteria based on clinical and laboratory features have been established (Table 354-5, HPIM-20).
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Sjögren's Syndrome
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