Treating Sjögrens Syndrome
The goals of treatment are to provide symptomatic relief as well as to reduce the risk of long-term damage. An assessment of the extent, activity, and aggressiveness of the disease process is the critical first step in determining the level of response.
Artificial tears, which vary in viscosity, are central to the management of xerophthalmia. Utilizing preparations with higher concentrations of hydroxymethylcellulose will increase the viscosity and therefore the duration of action. If the duration of action is still insufficient, an ophthalmologists assistance may be secured for punctal occlusion, first on a temporary basis to assess its efficacy, and then permanently by electrocautery. Artificial tears containing preservatives may cause a burning sensation. Preservative-free preparations are available.
Production of saliva may be stimulated by a variety of local and systemic measures. Sugar-free candies and gum may be used, though they may contain other carbohydrates that can still promote dental caries. Chewing on an inert substance such as paraffin or sucking on a fruit pit may also stimulate salivary flow. Sips of water are frequently sufficient to maintain oral moisture when symptoms are mild. Artificial saliva preparations are available. Given the dental risks in SS, special toothpastes have been developed for patients with xerostomia. These generate low levels of peroxide to augment the antibacterial effects of saliva. Topical fluoride preparations are indicated, particularly where the water supply is not fluoridated.
A number of systemic secretagogues have been evaluated. However, their effectiveness is dependent upon preservation of some residual glandular function. Both pilocarpine and cevimeline have been approved for use in the U.S. They may increase both tear and salivary flow in some individuals, but their usefulness is limited by tolerance to the side effects such as flushing and sweating.
Constitutional and musculoskeletal manifestations are treated with acetaminophen, salicylates, and non-steroidal anti-inflammatory agents (NSAIDs). NSAIDs must be used with care, given their risk for both GI and renal toxicity. Hydroxychloroquine has been useful in managing musculoskeletal manifestations and lymphadenopathy in some patients, though the results of controlled trials have been mixed. Corticosteroids are reserved for more serious or life-threatening major organ system involvement, such as central and peripheral nervous system involvement, pneumonitis, nephritis, hemolytic anemia, and vasculitis. Methotrexate, azathioprine, and cyclosporin A have been used for their corticosteroid-sparing effects. However, while there is frequently a reduction in the erythrocyte sedimentation rate and immunoglobulin levels, and the immunohistologic effects of immunosuppressive agents have also been demonstrated, the clinical effect upon sicca symptoms, arthralgias, and myalgias may be limited. Alkylating agents such as chlorambucil and cyclophosphamide should be used only with great caution, given the increased risk for the development of non-Hodgkins lymphoma in these patients.
Sjögrens syndrome may range in severity from a mild annoyance, through significant ocular and oral involvement with the potential for blindness and carious dental loss, respectively, to major organ system involvement. Rarely, however, is even major organ system involvement life-threatening.