Sjögren syndrome cannot be cured with currently available treatments. Instead, treatments primarily aim to minimize symptoms and prevent complications (Vivino 2016; Kurien 2017).
- Artificial tears and ophthalmic ointments. Topical therapy with artificial tears is an important component of first-line treatment for dry eyes (Saraux 2016). Artificial tears provide lubrication and add volume to the tear. Thicker preparations last longer but may blur vision. Non-irritating preservative-free solutions are preferred, especially when tears are used more than four times per day. Ophthalmic ointments are ideal for bedtime use since they remain on the eye surface longer than artificial tears (Rischmueller 2016).
- Cyclosporine eye drops. Topical application of eye drops containing cyclosporine, an immunosuppressant, twice daily is safe and effective for most Sjögren patients with eye dryness and inflammation. These eye drops can be used when eye lubricants are not sufficient (Mariette 2016; Ramos-Casals 2012).
- Tear duct plugs. Ophthalmologists may perform a relatively invasive procedure that involves insertion of plugs into tear duct openings to block drainage. These plugs, known as punctal plugs, provide dry eye relief by preserving natural and artificial tears. Punctal plugs may be temporary (collagen) or permanent (silicon), and are generally well tolerated. Adverse effects include overflow of tears, sensation that something is in the eye, and eye irritation (Rischmueller 2016; Ervin 2010; Chi 2012).
- Topical steroids. Corticosteroid eye drops, such as 0.1% clobetasone butyrate, can be used as a short-term (≤1 month), medically supervised therapy for moderate-to-severe dry eye symptoms (Aragona 2013).
- Salivary substitutes and stimulants. Important interventions for dry mouth include the use of salivary substitutes (artificial saliva) as a mouthwash to moisten oral tissues. Alternatively, specialized sugar-free chewing gums, fluoride gels, mouthwashes, prescription toothpastes, and lozenges containing agents that stimulate saliva production can be used to relieve dry mouth (Kurien 2017; Rischmueller 2016; Erlichman 1990; Konstantinidis 2007).
- Pilocarpine and cevimeline. Pilocarpine (Salagen, Pilopine) and cevimeline (Evoxac) are oral drugs indicated only when other therapies do not provide sufficient relief. They work by activating receptors for acetylcholine, a neurotransmitter that stimulates saliva production. Pilocarpine is associated with side effects such as nausea, flushing, sweating, and urinary frequency. Cevimeline activates receptors more selectively, but has similar side effects. Pilocarpine should be used with caution or avoided in patients with heart disease or respiratory disease due to risk of slow heart rate or bronchospasm, and in those taking blood pressure medications due to possible drug interactions (Kurien 2017; Rischmueller 2016; Takakura 2003).
Skin and Vaginal Dryness
- Lubricants and moisturizers. Lubricants and moisturizers can be used to treat skin and vaginal dryness (Rischmueller 2016).
Systemic Treatments for Severe Sjögren Syndrome
- Acetaminophen or nonsteroidal anti-inflammatory drugs for musculoskeletal symptoms. Acetaminophen (Tylenol) is a preferred treatment for musculoskeletal symptoms in Sjögren patients. Nonsteroidal anti-inflammatory drugs (NSAIDs) may also be used. NSAIDs include ibuprofen, naproxen, and diclofenac (Kurien 2017; NIH 2016a). However, caution should be exercised with frequent or long-term use of acetaminophen or NSAIDs. More information is available in the Acetaminophen and NSAID Toxicity protocol.
- Corticosteroids. Corticosteroids are sometimes used to treat acute flare-ups of inflammatory arthritis (Kurien 2017; Vivino 2016).
- Hydroxychloroquine. Hydroxychloroquine is an immunosuppressive drug used to treat inflammatory musculoskeletal pain in primary Sjögren syndrome. Hydroxychloroquine may also be used to treat fatigue in some Sjögren patients. Regular monitoring of the eyes for retinal toxicity is required (Vivino 2016; Mariette 2016; Geamanu Panca 2014; Espandar 2016).
- Other disease-modifying antirheumatic drugs. If hydroxychloroquine is ineffective for inflammatory musculoskeletal pain, other disease-modifying antirheumatic drugs may be considered. Options include methotrexate, leflunomide (Arava), sulfasalazine (Azulfidine), azathioprine (eg, Imuran), and cyclosporine (eg, Restasis) (Vivino 2016).
- Cyclophosphamide. Cyclophosphamide is an immunosuppressive drug that has been shown to be effective in the treatment of spinal cord compression disorders associated with autoimmune diseases, including Sjögren syndrome (Valim 2015; Hamming 2015; de Seze 2006). In a study in Sjögren patients with severe neurological symptoms due to spinal cord compression, treatment with monthly infusions of cyclophosphamide together with corticosteroids markedly improved several measures of disability (de Seze 2006; Hamming 2015).
- Rituximab (Rituxan). Rituximab is a monoclonal antibody that may be a useful therapeutic option for cases of persistent swelling of the salivary glands or systemic complications. It works by depleting B cells in the blood. Sjögren patients taking rituximab require close monitoring of possible significant toxic side effects (Vivino 2016; Mariette 2016; Pers 2007).