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With the exception of pilocarpine and cevilemine to treat dry mouth symptoms, there are no medications that have been approved by the U.S. Food and Drug Administration (FDA) for treatment of severe glandular or extraglandular symptoms associated with Sjogren's syndrome. Disease modifying antirheumatic drugs (DMARDs) such as those used for rheumatoid arthritis and systemic lupus erythematosus (SLE) have had limited success in the treatment of severe primary Sjogren's syndrome though they may be effective on a limited basis in managing extraglandular manifestations. Medications to treat Sjogren's syndrome and the systemic manifestations are chosen on the basis of what seems to work for other patients and are modified on an as-needed basis.

In general, medications used for the treatment of severe Sjogren's syndrome and various systemic manifestations of Sjogren's syndrome include:

• Anti-inflammatory drugs, such as aspirin, Motrin or Advil. Some of these medications may be administered as a topical cream or suppository for patients who have difficulty swallowing pills.

• Immunosuppressive drugs

  • corticosteroids (e.g., Prednisone). Use of corticosteroids is limited by their side effects, including: osteoporosis, diabetes, mood changes, and cardiovascular changes. In addition, they accelerate the development of periodontal disease and oral candidiasis.
  • methotrexate (Trexall)
  • azathioprine (Imuran) - studies regarding the efficacy of this drug have so far been inconclusive.
  • cyclophosphamide (Cytoxan) - an alkylating agent used to treat many types of cancer. In Sjogren's syndrome, it may be used for potentially life threatening conditions but it is used with caution due to the high risk of lymphoma. To minimize this risk, some clinicians recommend pulse treatment rather than daily administration.
  • cyclosporine

• Disease Modifying Antirheumatic Drugs (DMARDs)

  • hydroxychloroquine (Plaquenil) - regular eye checkups are recommended when taking this drug since an uncommon side effect is accumulation of this drug in the eye.
  • leflunomide

• Biological agents (immunomodifying drugs)

  • infliximab (Remicade) - this is a recombinant anti-tumor necrosis factor monoclonal antibody. To date, data regarding the efficacy of infliximab have been inconclusive, with some small studies indicating significant improvement of symptoms while a 2004 multicenter, randomized, double-blind study clearly showed a lack of efficacy. It is not used as a first-line treatment but may be effective for some types of severe, refractory extraglandular features of Sjogren's syndrome.
  • rituximab (Rituxan) - this is an anti-CD20 monoclonal antibody that appears of have an effect on B-cell hyperactivation which is closely associated with Sjogren's syndrome and with development of B-cell lymphoma.
  • interferon-alpha - improved salivary output and decreased lymphocytic infiltration have been reported in some studies.
  • etanercept (Enbrel) - data regarding the efficacy of etanercept is not conclusive.
  • eculizumab (Soliris)

• Other medications:

  • thalidomide (Thalomid)
  • dehydroepiandrosteron (DHEA)
  • octreotide (Sandostatin)
  • antiviral agents
  • vitamin b12

Biological Agents

Biological agents target specific factors in the autoimmune response, such as B-cells and T-cells, and are a major advance for the treatment of glandular and extraglandular symptoms in patients with Sjogren's syndrome. Anti-B-cell medications have not been approved by the U.S. Food and Drug Administration (FDA) at this time. They are used for patients with severe cases of Sjogren's syndrome who are refractory to standard therapy of corticosteroids and other immunosuppressive agents or patients with a life threatening situation. The overall long-term risk of biological agents is unknown. Rituximab was approved in 1997 to treat B-cell lymphoma. It is also used to treat various autoimmune disorders such as rheumatoid arthritis.