26th January 2024

Management

The goals of management necessitate a multi-disciplinary team approach through pre-treatment evaluation, which allows tailored treatment to individual patients and their particular disease manifestations. 

Non-pharmacologic interventions 

• Self-management of sicca symptoms using artificial tears and artificial saliva. 
• Immunisations: Appropriate immunisations should be received prior to the start of immunosuppressive therapy, for example, COVID-19, herpes zoster and pneumococcal vaccinations. 
• Smoking cessation: This is of particular importance as smoking cigarettes or cannabis can cause worsening symptoms of dry eyes and mouth, increase the risk of dental complications and reduce the efficacy of medications such as hydroxychloroquine. 

Pharmacological Interventions: 

• Lubricating ocular ointments such as topical Cyclosporine or Lifitegrast and oral secretagogues such as Pilocarpine or cevimeline may be used to treat moderate to severe sicca symptoms. 
• Glucocorticoids can be used to treat systemic manifestations of SS in a similar way to other autoimmune disease. However, weaning of steroids is necessary as long-term corticosteroid use is associated with many adverse effects. 
• Immunosuppressive and DMARDs: 
  • Hydroxychloroquine can be used to improve symptoms such as arthralgias and myalgias. 
  • Methotrexate is effective in controlling dry mouth and eyes, arthralgias, arthritis and musculoskeletal pain. 
  • Azathioprine at high dose can be used to manage extra-glandular manifestations such as interstitial pneumonitis, myelopathy and autoimmune hepatitis. 
  • Mycophenolate mofetil has been shown to lead to a reduction in hypergammaglobulinemia and rheumatoid factor and an increase in complement and white blood cell count, making it effective in treating agranulocytosis as a result of primary SS.