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Therapeutic Approach

Sjögren’s syndrome is a multisystem autoimmune disease that primarily affects the lacrimal and salivary glands. Patients typically present with oral and ocular sicca symptoms. Systemic involvement (extraglandular manifestations) occurs in 30%-50% of patients and include involvement of the skin, lungs, GI tract, nervous system, kidneys, and the musculoskeletal system. Lymphoma is a recognized complication. Sjögren’s syndrome is categorized as primary when it presents in the absence of another rheumatologic disease, and updated classification criteria have been published recently.1 Disease activity can be captured by the ESSDAI2 and ESSPRI.3 Dermatologic manifestations of Sjögren’s syndrome include but are not limited to xerosis, leukocytoclastic vasculitis, cryoglobulinemic vasculitis, annular erythema, and subcutaneous amyloid. Therapy is primarily focused on controlling sicca symptoms with oral and topical agents. Extraglandular manifestations can be managed with immunosuppressive medications.

Rescue therapies such as punctal plugs and amniotic membrane transplant may be considered in consultation with ophthalmology though the evidence supporting these is limited. See Table 55-1.

Table 55-1Sjögren’s Syndrome Treatment Table

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