What is Sjögren's Syndrome?
Sjögren's syndrome is an autoimmune condition characterized by dry mouth, dry eyes, rash, blood cell abnormalities, and Raynaud’s phenomenon (pain and color changes in the hands). It has a close association with thyroid disease, but it is unclear whether they have a direct relationship. Patients can also develop joint pains, pulmonary fibrosis, peripheral neuropathy, and mild anemia.
Patients with Sjögren's syndrome have a markedly increased risk of developing lymphomas compared to healthy controls. Due to the importance of this condition, it is critical that patients understand its causes, symptoms, and treatment.
What Causes Sjögren's Syndrome?
The etiology of Sjögren's syndrome is not known, but the condition is believed to originate from genetic and environmental factors. Sjögren's syndrome is an autoimmune disorder – these types of disorders are characterized by the inappropriate attack of normal tissue by the immune system. Patients with the disease typically develop autoimmune injury to structures such as the salivary glands, lacrimal glands, and small blood vessels. Certain autoantibodies such as anti-Ro/SSA and anti-La/SSB are closely associated with the disease.
About 10%-70% of patients with Sjögren's syndrome also have evidence of autoimmune thyroid disease. Sjögren's syndrome is also associated with rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis.
How Common is Sjögren's Syndrome?
Sjögren's syndrome is an uncommon disorder. The condition often requires referral to a rheumatology specialist, especially if the diagnosis is in question or first-line measures are unsuccessful.
Only about 10% of patients with dry eyes have Sjögren's syndrome. The incidence of the condition is about 4 per 100,000 people per year. The prevalence of the disease is estimated to be approximately 0.043%.
Signs and Symptoms
Symptoms and signs of Sjögren's syndrome often include:
- Dry mouth - xerostomia
- Dental cavities – dental caries
- Dry eye syndrome – keratoconjunctivitis sicca
- Purpuric rash
- Raynaud’s phenomenon
- Joint and muscle pain - arthralgia and arthritis
- Interstitial lung disease - shortness of breath & cough
- Peripheral neuropathy – numbness, tingling, paresthesias
- Blood cell manifestations – anemia, leukopenia, cryoglobulinemia, and lymphoma
Raynaud’s phenomenon is a condition where the fingers become painful and changes colors (eg, white, blue) in cold temperature – this occurs due to constriction of the blood vessels in the digits of the hand.
The diagnosis of Sjögren's syndrome is suggested based on symptoms and physical examination, but typically confirmed with laboratory studies. Specific serologic blood tests include anti-Ro/SSA and anti-La/SSB antibodies. Your doctor may also check an antinuclear-antibody (ANA) and rheumatoid factor (RF).
Your doctor may perform a Schirmer test to evaluate for dry eye. They may also conduct tests for decreased secretion of saliva (salivary hypofunction).
Occasionally, they will recommend magnetic resonance imaging (MRI) or ultrasound (US) to evaluate for glandular abnormalities. They may also want to obtain a lip biopsy evaluating for autoimmune salivary gland inflammation (lymphocytic sialadenitis).
Commonly ordered blood tests include a CMP (comprehensive metabolic panel), CBC (complete blood cell count), and thyroid function (TSH, free T4).
Sjögren's Syndrome Medication
Patients are usually instructed on basic oral health measures such proper brushing and flossing, good hydration, avoiding beverages with sugar or additives, and using sugar-free lozenges or other salivary stimulants. They should also avoid medications that can contribute to dry mouth such as tricyclic antidepressants and antihistamines. Patients with Sjögren's syndrome should also see their dentist regularly.
Some can benefit from temporary use of artificial saliva – this typically contains a mix of multiple ingredients such as carboxymethylcellulose, polyethylene glycol, and sorbitol. Patients with symptoms that are refractory to conservative measures may benefit from a muscarinic agonist. Options typically include Salagen (Pilocarpine) or Evoxac (cevimeline). There is some evidence to suggest that Plaquenil (Hydroxychloroquine) is beneficial for dry mouth symptoms as well.
Patients with eye disease are commonly started on artificial tears and lubricants. Some patient will benefit from topical Restasis (Cyclosporine) or Xiidra (Lifitegrast). Topical corticosteroids are occasionally necessary for several weeks. All of these topical eye therapies should be prescribed by an ophthalmology specialist.
- Mavragani CP, Nezos A, Moutsopoulos HM. New advances in the classification, pathogenesis, and treatment of Sjogren's syndrome. Curr Opin Rheumatol 2013; 25:623. - https://www.ncbi.nlm.nih.gov/pubmed/23846338
- Ramos-Casals M, Tzioufas AG, Font J. Primary Sjögren's syndrome: new clinical and therapeutic concepts. Ann Rheum Dis 2005; 64:347. - https://ard.bmj.com/content/annrheumdis/64/3/347.full.pdf
- Ramos-Casals M, Tzioufas AG, Stone JH, et al. Treatment of primary Sjögren syndrome: a systematic review. JAMA 2010; 304:452. - https://jamanetwork.com/journals/jama/fullarticle/186274
The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.