Treatment Options for Sjogren's Syndrome

Prognosis in Sjogren's Syndrome

Sjogren's syndrome is typically a benign process but has a significant impact on quality of life. Most dry mouth/eyes symptoms and musculoskeletal symptoms such as arthralgia and fatigue can be managed effectively with muscarinic agonists, corticosteroids, and several self-help techniques. Systemic therapy including traditional antirheumatic drugs to alter the course of the disease and halt the decline of glandular function has not been effective. Regardless of treatment, though, salivary flow is lost over time and many patients will experience at least one episode of inflammatory arthritis in the course of the disease.

There is a subset of patients who develop severe sicca symptoms and extraglandular involvement. These patients require continued monitoring and aggressive treatment that may consist of corticosteroids, immunosuppressive agents, biological agents, and/or plasmapheresis. Aggressive therapy and monitoring is particularly important for patients whose symptoms and blood values place them at high risk for the development of lymphoma. Approximately 60% of patients exhibit antibodies against Ro/SS-A and La/SS-B. The presence of these antibodies is associated with:

  • Early disease onset
  • Longer disease duration
  • Parotid gland enlargement
  • Frequency of extraglandular involvement
  • Greater infiltration of lymphocytes into the glands

Although patients with primary Sjogren's syndrome do not overall have a higher mortality rate as compared to the general population, they are at higher risk of dying from a lymphoproliferative condition such as lymphoma which develops in approximately 5% of patients with primary Sjogren's syndrome. The most common types of lymphoma associated with Sjogren's disease are low-grade, marginal zone B-cell lymphoma and MALT, and are typically located in the parotid gland. Lymphoma is usually treated with rituximab and other standard chemotherapy treatment protocols.

An adverse outcome for Sjogren's syndrome is associated with four factors present at diagnosis which require intensive monitoring, namely:

  • Vasculitis
  • Grades III-IV parotid scintigraphy
  • Hypocomplementemia (low C3/C4 levels in the blood)
  • Cryoglobulinemia (low levels of cryoglobulins in the blood)

For further information about prognosis in Sjogren's syndrome, please click on the following link: http://www.medifocus.com/abstracts.php?gid=RH011&ID=17569749