What is the primary management approach for patients with sicca (Sjögren's) syndrome?

From the Guidelines

The primary management approach for patients with sicca (Sjögren's) syndrome focuses on symptom relief and prevention of complications, with the most recent evidence from 2024 suggesting a comprehensive approach that includes aggressive moisture replacement therapy, oral secretagogues, and meticulous oral hygiene 1.

Key Components of Management

• Aggressive moisture replacement therapy for dry eyes and mouth
• Oral secretagogues, such as pilocarpine or cevimeline, to stimulate saliva production
• Meticulous oral hygiene, including regular dental visits, daily fluoride treatments, and avoiding mouth-drying substances
• Topical lubricants, topical anti-inflammatory therapy, and tear-conserving strategies for dry eye syndrome
• Consideration of systemic manifestations, such as fatigue and joint pain, and potential use of hydroxychloroquine or immunosuppressants

Treatment Options

• Artificial tears (preservative-free formulations like Refresh or Systane) for dry eyes
• Punctal plugs to reduce tear drainage
• Sugar-free lozenges or gum to stimulate saliva, and artificial saliva substitutes like Biotène for dry mouth
• Oral pilocarpine (5-10 mg three times daily) or cevimeline (30 mg three times daily) to stimulate saliva production
• Hydroxychloroquine (200-400 mg daily) for systemic manifestations, such as fatigue and joint pain

Important Considerations

• Patients with Sjögren's syndrome should be comanaged with a rheumatologist due to potential systemic complications 1
• Severe sicca syndrome can result in dental caries and loss of teeth if left untreated 1
• Autoantibodies are often absent in patients with CPI-induced sicca syndrome, and diagnosis should be based on clinical presentation and objective parameters of tear film stability and ocular surface damage 1

From the FDA Drug Label

The recommended dose of pilocarpine hydrochloride tablets is one tablet (5 mg) taken four times a day. Efficacy was established by 6 weeks of use. The primary management approach for patients with Sicca (Sjögren's) syndrome is the use of pilocarpine hydrochloride tablets at a dose of 5 mg four times a day 2.

• The goal of this treatment is to provide relief from dry mouth symptoms.
• Efficacy was established after 6 weeks of use.
• It is essential to note that the treatment should be tailored to the individual patient's response and tolerability.
• The most common adverse events associated with pilocarpine hydrochloride tablets in Sjögren's syndrome patients include sweating, urinary frequency, chills, and vasodilatation (flushing) 2.

From the Research

Primary Management Approach for Sicca Syndrome

The primary management approach for patients with sicca (Sjögren's) syndrome is mainly symptomatic, primarily directed against sicca complaints 3.

Symptomatic Relief

• Nonpharmacologic measures to preserve secretions, and tear and saliva substitutes, offer some symptomatic relief for dry eyes and mouth 3.
• Muscarinic agonists and topical cyclosporine yield well-documented improvement in ocular sicca features 3.
• Pilocarpine hydrochloride has been proven to be efficacious in treating dry mouth and dry eye symptoms in Sjögren's syndrome patients 4, 5.

Treatment of Extraglandular Disease

• For the potential severe, nonexocrine manifestations complicating pSS, standard high-dose immunosuppression is used 3.
• B cell depleting treatments and other newer biologic therapies appear more promising for treating extraglandular disease 3.

Interdisciplinary Evaluation

• A comprehensive evaluation of oral and ocular symptoms and findings in primary Sjögren's syndrome (pSS) patients may provide valuable information for management 6.
• The ESSPRI index is useful in evaluating health-related quality of life in primary SS patients, particularly in assessing the intensity of oral dryness 7.

Factors Influencing Dry Mouth

• Oral dryness correlates with age, quality of sleep, anxiety, depression, and vascular risk factors such as hypertension, type II diabetes mellitus, and hypercholesterolemia 7.
• A multidisciplinary therapeutic approach may be the best way of minimizing oral dryness and its consequences in primary SS patients 7.