What is the treatment for Sicca (Sjögren's) syndrome?

From the Guidelines

The treatment for Sicca (Sjögren's) syndrome should focus on symptom management and preventing complications, with the first therapeutic approach for dryness being symptomatic relief using topical therapies, as recommended by the EULAR guidelines 1. The management of Sjögren's syndrome requires a multidisciplinary approach, involving various health professionals, with a central role for specialists in autoimmune diseases.

• The first-line therapeutic approach to ocular dryness includes the use of artificial tears and ocular gels/ointments, with preservative-free options preferred for frequent use.
• For dry mouth, patients should sip water frequently, use sugar-free gum or lozenges to stimulate saliva, and consider saliva substitutes like Biotene.
• Pilocarpine (5-10mg three times daily) or cevimeline (30mg three times daily) can stimulate saliva production, as supported by the study on dry eye syndrome preferred practice pattern 1.
• Systemic symptoms, such as fatigue and joint pain, may be managed with hydroxychloroquine (200-400mg daily), although the evidence for its efficacy in dry eye is not strong, as noted in the outcomes-based review of treatment options for patients with dry eye secondary to Sjögren's syndrome 1.
• More severe cases might require immunosuppressants like methotrexate, rituximab, or corticosteroids, with the treatment of systemic disease tailored to organ-specific severity using the ESSDAI definitions, as recommended by the EULAR guidelines 1.
• Patients should maintain good oral hygiene with regular dental visits, avoid smoking, limit alcohol and caffeine consumption, and use humidifiers in dry environments, as part of a comprehensive management plan. The use of systemic therapies, such as glucocorticoids and immunosuppressive agents, should be restricted to patients with active systemic disease, with the goal of minimizing the risk of adverse events and maximizing the benefits of treatment, as emphasized in the EULAR recommendations 1.

From the FDA Drug Label

Pilocarpine hydrochloride tablets are indicated for ... the treatment of symptoms of dry mouth in patients with Sjogren’s Syndrome. 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 treatment for Sicca (Sjögren's) syndrome is pilocarpine (PO), with a recommended dose of 5 mg taken four times a day 2 2.

• The lowest effective dose should be used for maintenance.
• Efficacy was established by 6 weeks of use.

From the Research

Treatment Options for Sicca (Sjögren's) Syndrome

The treatment for Sicca (Sjögren's) syndrome focuses on alleviating the symptoms of dry mouth and dry eyes. Several studies have investigated the efficacy of various treatments, including:

• Pilocarpine hydrochloride: A muscarinic cholinergic agonist that has been shown to improve dry mouth and dry eye symptoms in patients with Sjögren's syndrome 3, 4, 5.
• Topical therapies: Saliva substitutes and preservative-free artificial tears can provide symptomatic relief for dry mouth and dry eyes 6.
• Sialogogues: Cevimeline is another sialogogue that can stimulate salivary flow in patients with residual salivary gland function 6.
• Topical cyclosporine A: May be used in severe cases of keratoconjunctivitis sicca 6.
• Hydroxychloroquine: May be appropriate for patients with fatigue, arthralgia, and myalgia 6.
• Glucocorticoids and immunosuppressive agents: Should be reserved for severe systemic involvement 6.
• B-cell depleting agents: Rituximab has shown promise in treating primary Sjögren's syndrome, particularly in patients with early active disease or extraglandular involvement 7.

Efficacy of Pilocarpine

Studies have demonstrated the efficacy of pilocarpine in treating dry mouth and dry eye symptoms in patients with Sjögren's syndrome. For example:

• A study published in 1997 found that oral pilocarpine hydrochloride (9 mg/day) improved dry mouth and dry eye symptoms in 53% and 26% of patients, respectively 3.
• A randomized, placebo-controlled study published in 2004 found that oral pilocarpine (20-30 mg/day) significantly increased salivary flow and improved dry mouth and dry eye symptoms in patients with Sjögren's syndrome 4.
• A prospective study published in 2015 found that oral pilocarpine improved subjective eye symptoms, fluorescein staining scores, and tear film breakup time measurements in patients with severe dry eye unresponsive to conventional conservative treatment 5.