Sjögren's Syndrome

How Sjögren's develops

White blood cells infiltrate the lacrimal (tear) and salivary glands and gradually reduce their output. Autoantibodies such as anti-SSA (Ro) and anti-SSB (La) are often present. The immune activity can also affect other organs.

• Primary Sjögren's occurs on its own.
• Secondary (associated) Sjögren's occurs with another autoimmune disease, most often rheumatoid arthritis or lupus.

Symptoms

Dryness (sicca) symptoms

• eyes: gritty, sandy or burning feeling, redness, light sensitivity, blurred vision that clears with blinking (dry eye)
• mouth: dryness, needing water to swallow dry food, difficulty speaking for long, altered taste, cracked lips, oral thrush (dry mouth)
• teeth: rapid decay, especially at the gumline (dental caries)
• nose, throat and voice: dryness, hoarseness, nosebleeds, dry cough
• skin and vagina: dryness, itch, painful intercourse

Glandular swelling

Recurrent swelling of the parotid glands in front of the ears or the glands under the jaw (parotitis).

Whole-body (systemic) features

• profound fatigue
• joint and muscle pain
• peripheral neuropathy: numbness, tingling, burning feet
• lung involvement: dry cough, interstitial lung disease
• kidney involvement, including kidney tubule problems
• vasculitis rash on the legs
• Raynaud's phenomenon
• low thyroid function (often coexisting autoimmune thyroid disease)
• brain fog

Diagnosis

There is no single test. Diagnosis combines:

• blood tests: anti-SSA/Ro (and sometimes anti-SSB/La), ANA, rheumatoid factor, immunoglobulins, complement levels, blood count
• Schirmer test: a paper strip measures tear production
• ocular staining: dyes show damage to the eye surface
• unstimulated salivary flow: measures saliva over a set time
• minor salivary gland (lip) biopsy: shows clusters of immune cells (focus score)
• salivary gland ultrasound

The ACR/EULAR classification criteria use a score based on lip biopsy, anti-SSA antibodies, ocular staining, Schirmer test and salivary flow.

Treatment

Dry eyes

• preservative-free artificial tears and gels, ointments at night
• prescription anti-inflammatory drops (cyclosporine, lifitegrast)
• punctal plugs to keep tears on the eye
• humidifiers, moisture goggles, screen breaks

Dry mouth

• frequent sips of water, sugar-free gum or lozenges with xylitol
• saliva substitutes
• pilocarpine or cevimeline tablets to stimulate saliva (and tears)
• avoid alcohol-containing mouthwashes, smoking and drying medicines where possible

Dental care

• prescription-strength fluoride toothpaste or varnish
• dental checkups every 3 to 6 months
• treat oral thrush promptly

Systemic disease

• hydroxychloroquine for joint pain, fatigue and rash
• NSAIDs for joint pain, following the label
• corticosteroids, methotrexate, mycophenolate, azathioprine or rituximab for organ involvement under rheumatology care

Complications

• lymphoma: people with Sjögren's have a higher risk of non-Hodgkin lymphoma, especially with persistent gland swelling, low complement levels or vasculitis
• corneal ulcers and infections
• tooth loss
• lung, kidney and nerve damage
• in pregnancy, anti-SSA antibodies can rarely cause congenital heart block or neonatal lupus in the baby, so pregnancies are monitored

Outlook

Sjögren's is usually a chronic condition with stable symptoms. Many people manage well with dryness treatments. Those with systemic involvement need regular rheumatology follow-up. Life expectancy is generally normal unless serious complications such as lymphoma occur.

Frequently asked questions

What are the first symptoms of Sjögren's syndrome?

The most common first symptoms are dry, gritty eyes and a dry mouth that makes swallowing dry food difficult. Fatigue, joint pain, swollen salivary glands and frequent dental cavities are also common early features.

How is Sjögren's syndrome diagnosed?

Doctors combine blood tests for anti-SSA (Ro) antibodies, tests of tear and saliva production, an eye surface staining test, and sometimes a lip biopsy that shows immune cell clusters in the salivary glands.

Is Sjögren's syndrome serious?

For many people it mainly causes uncomfortable dryness and fatigue. Some develop involvement of the lungs, kidneys, nerves or blood vessels, and there is a higher risk of lymphoma. Regular follow-up helps detect and treat these problems early.

Can Sjögren's syndrome be cured?

There is no cure, but treatments relieve dryness, protect the eyes and teeth, and control inflammation. Medicines such as hydroxychloroquine and immunosuppressants are used when other organs are involved.