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  • • Chronic, systemic inflammatory disorder of unknown etiology

    • Dry mouth, eyes, and other mucous membranes

    • Associated with rheumatic disorders such as rheumatoid arthritis, scleroderma, and systemic lupus erythematosus (SLE) (all have lymphocytic infiltration into affected tissues)

    • Association with HLA-DR3

    • Primary syndrome involves the eyes, mouth, and mucous membranes (sicca syndrome or complex); secondary syndrome has an associated generalized collagen-vascular disease

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Epidemiology

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  • • More common than SLE, but less common than rheumatoid arthritis

    • Arthritis occurs in about 33% of patients

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Symptoms and Signs

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  • • Dessicated cornea and conjunctiva

    • 33% of patients have enlarged parotid glands—usually firm, smooth, mildly tender, and fluctuate in size

    • Taste and smell sensations diminished

    • May have dry skin

    • Dry mucous membranes throughout the body

    • Alopecia

    • Joint inflammation

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Laboratory Findings

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  • • Schirmer test: Measures quantity of tears secreted in 5 minutes in response to irritation stimuli; decreased in Sjögren syndrome

    • Evaluate salivary glands by salivary flow, sialography, salivary scintiscan, or biopsy

    • Elevated levels of serum antibodies to gamma globulin, nuclear protein, and many tissue constituents

    • Elevated rheumatoid factor (70% of cases)

    • Negative VDRL

    • Elevated ESR (70% of cases)

    • Proteinuria (in presence of interstitial nephritis)

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  • • Dryness of the respiratory tract may lead to lung infections

    • Associated with chronic hepatobiliary disease and pancreatitis

    • May develop fibrinus pericarditis, sensory neuropathy, and renal insufficiency (due to interstitial nephritis)

    • Patients at risk for Waldenstrom macroglobulinemia

    • Slit lamp exam is useful to evaluate for Sjögren syndrome

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Rule Out

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  • • Lymphoma (44-fold increased risk for lymphoma in patients with Sjögren syndrome)

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  • • History and physical exam suggests diagnosis

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Surgery

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Indications

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  • • Tarsorrhaphy if artificial tears fail

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Medications

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  • • Artificial tears

    • Saliva substitute, chewing sugar-free gum, and sipping fluids throughout the day

    • Oral corticosteroids or immunosuppressants are rarely indicated as connective tissue involvement is usually mild and chronic

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Prognosis

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  • • Related to the underlying connective tissue disorder

    • Death rarely occurs from pulmonary failure, renal failure, or lymphoma

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  • • Belafsky PC, Postma GN. The laryngeal and esophageal manifestations of Sjögren's syndrome. Curr Rheumatol Rep. 2003;5:297.

    • Borchers AT et al. Immunopathogenesis of Sjögren's syndrome. Clin Rev Allergy Immunol. 2003;25:89.

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