Primary Sjogren Syndrome

Not yet reviewed

This information is AI generated and has not yet been reviewed by a specialist physician. AI can make mistakes.

Disease overview

Chronic autoimmune disorder heavily predominant in women which primarily targets the salivary and lacrimal glands, but is capable of causing systemic symptoms [more]. The primary disease occurs alone (compared to secondary Sjogren Syndrome, seen in the setting of other autoimmune diseases).

Common symptoms

Classic symptoms include dry eyes and mouth secondary to autoimmune epithelitis of these exocrine glands (sicca syndrome) [ ]. Lymphocytic infiltration and the presence of immune complexes can affect many other systemscommon additional symptoms include fatiguearthralgianeuropathydysphagiaand .

When to suspect

  • Recommendation 1

    PSS is primarily a clinical diagnosis, though presence of Anti-Ro/SSA and/or Anti-La/SSB autoantibodies are strongly associated with disease (specific, though not sensitive). Multiple classification criteria are available to guide clinicians and improve accuracy of assessment.

How to test

  • Recommendation 1

    PSS is primarily a clinical diagnosis, though presence of Anti-Ro/SSA and/or Anti-La/SSB autoantibodies are strongly associated with disease (specific, though not sensitive). Multiple classification criteria are available to guide clinicians and improve accuracy of assessment.

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Treatment

  • Recommendation 1

    Disease-specific treatment is limited. Sicca symptoms may be managed topically (artificial tears, saliva substitutes)

  • Recommendation 2

    systemic symptoms are usually treated with immunosuppression (such as methotrexate

  • Recommendation 3

    IV steroids may be needed for severe symptoms) [more]. Oral muscarinic agents may help sicca symptoms as well

  • Recommendation 4

    hydroxychloroquine is a recommended agent for moderate systemic disease [more].

Primary care

  • Recommendation 1

    PSS is a chronic disease and may require treatment modifications over time, particularly as research is ongoing for more targeted therapies. Patients are at increased risk for non-Hodgkin lymphoma due to chronic overactivation of B-cells [more].

Further support

  • Recommendation 1

    Depending on each patient's presenting symptoms, a multi-disciplinary team may be necessary. A large number of academic medical centers across the U.S. have a dedicated Sjogren's Syndrome program. Referral to Medical Genetics Department, if available. Initial virtual care is also available through organizations like TeleRare Health.

Clinical trials

Clinical Trials

Sources

No data available

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