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| Disease found: | Primary Sjogren Syndrome |
| Current as of: | October 8, 2024 |
| 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). |
| Signs and Symptoms: | Classic symptoms include dry eyes and mouth secondary to autoimmune epithelitis of these exocrine glands (sicca syndrome) [more]. Lymphocytic infiltration and the presence of immune complexes can affect many other systems; common additional symptoms include fatigue, arthralgia, neuropathy, dysphagia, and more. |
| Diagnosis: | 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. |
| Treatment: | Disease-specific treatment is limited. Sicca symptoms may be managed topically (artificial tears, saliva substitutes); systemic symptoms are usually treated with immunosuppression (such as methotrexate; IV steroids may be needed for severe symptoms) [more]. Oral muscarinic agents may help sicca symptoms as well; hydroxychloroquine is a recommended agent for moderate systemic disease [more]. |
| Clinical Management: | 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]. |
| Referral: | 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: | A large number of studies are currently recruiting globally. |