Sjögren syndrome

Definition

Sjögren syndrome is an autoimmune disorder in which the glands that produce tears and saliva are destroyed. This causes dry mouth and dry eyes. The condition may affect other parts of the body, including the kidneys and lungs.

Alternative Names

Xerostomia - Sjögren syndrome; Keratoconjunctivitis sicca - Sjögren; Sicca syndrome; Sjögren syndrome

Causes

The cause of Sjögren syndrome is unknown. It is an autoimmune disorder. This means the body attacks healthy tissue by mistake. The syndrome occurs most often in women ages 40 to 50. It is rare in children.

Primary Sjögren syndrome is defined as dry eyes and dry mouth without another autoimmune disorder.

Secondary Sjögren syndrome occurs along with another autoimmune disorder, such as:

• Rheumatoid arthritis (RA)
• Systemic lupus erythematosus
• Scleroderma
• Polymyositis
• Hepatitis C can affect the salivary glands and looks like Sjögren syndrome
• IgG4 disease can look like Sjogren syndrome and should be considered

Symptoms

Dry eyes and dry mouth are the most common symptoms of this syndrome.

Eye symptoms:

• Itching eyes
• Burning sensation in eyes
• Feeling that something is in the eye

Mouth and throat symptoms:

• Difficulty swallowing or eating dry foods
• Loss of sense of taste
• Problems speaking
• Thick or stringy saliva
• Mouth sores or pain
• Teeth decay and gum inflammation
• Hoarseness

Other symptoms may include:

• Fatigue
• Fever
• Change in the color of hands or feet with cold exposure (Raynaud phenomenon)
• Joint pain or joint swelling
• Swollen glands
• Skin rash
• Numbness and pain due to neuropathy
• Cough and shortness of breath due to lung disease
• Irregular heartbeat
• Nausea and heartburn
• Vaginal dryness or painful urination

Exams and Tests

A complete physical exam will be done. The exam reveals dry eyes and dry mouth. There may be mouth sores, decayed teeth or gum inflammation. This occurs because of the mouth dryness. Your health care provider will look in your mouth for fungus infection (candida). Skin may show a rash, the lung exam may be abnormal, the abdomen will be palpated for liver enlargement. The joints will be examined for arthritis. The neuro exam will look for deficits.

You may have the following tests done:

• Complete blood chemistry with liver enzymes
• Complete blood count with differential
• Urinalysis
• Antinuclear antibodies (ANA) test
• Anti-Ro/SSA and anti-La/SSB antibodies
• Rheumatoid factor
• Test for cryoglobulins
• Complement levels
• Protein electrophoresis
• Test for hepatitis C and HIV (if at risk)
• Thyroid tests
• Schirmer test of tear production
• Imaging of the salivary gland: by ultrasound or by MRI
• Salivary gland biopsy
• Skin biopsy if a rash is present
• Examination of the eyes by an ophthalmologist
• Chest x-ray

Treatment

The goal is to relieve symptoms.

• Dry eyes may be treated with artificial tears, eye-lubricating ointments, or cyclosporine liquid.
• If Candida is present, it may be treated with sugar-free miconazole or nystatin preparations.
• Tiny plugs can be placed in the tear drainage ducts to help the tears stay on the surface of the eye.

Disease-modifying antirheumatic drugs (DMARDs) similar to those used for RA may improve the symptoms of Sjögren syndrome. These include tumor necrosis factor (TNF) inhibiting drugs such as Enbrel, Humira or Remicaide.

Some things you can do to ease symptoms include:

• Sip water throughout the day
• Chew sugarless gum
• Avoid medicines that can cause mouth dryness, such as antihistamines and decongestants
• Avoid alcohol

Talk with your dentist about:

• Mouth rinses to replace minerals in your teeth
• Saliva substitutes
• Drugs that help your salivary glands make more saliva

To prevent dental decay caused by mouth dryness:

• Brush and floss your teeth often
• Visit the dentist for regular checkups and cleanings

Outlook (Prognosis)

The disease is most often not life threatening. The outcome depends on what other diseases you have.

There is a higher risk for lymphoma and early death when Sjögren syndrome has been very active for a long time, as well as in people with vasculitis, low complements, and cryoglobulins.

Possible Complications

Complications may include:

• Damage to the eye
• Dental cavities
• Kidney failure (rare)
• Lymphoma
• Pulmonary disease
• Vasculitis (rare)
• Neuropathy
• Bladder inflammation

When to Contact a Medical Professional

Call your provider if you develop symptoms of Sjögren syndrome.

References

Baer AN, Alevizos I. Sjögren syndrome. In: Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology. 7th ed. Philadelphia, PA: Elsevier; 2019:chap 147.

Mariette X, Nocuturne C. Sjogren syndrome. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 252.

Seror R, Bootsma H, Saraux A, et al. Defining disease activity states and clinically meaningful improvement in primary Sjögren's syndrome with EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI). Ann Rheum Dis. 2016;75(2):382-389. PMID: 25480887 pubmed.ncbi.nlm.nih.gov/25480887/.

Singh AG, Singh S, Matteson EL. Rate, risk factors and causes of mortality in patients with Sjögren's syndrome: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford). 2016;55(3):450-460. PMID: 26412810 pubmed.ncbi.nlm.nih.gov/26412810/.

Turner MD. Oral manifestations of systemic diseases. In: Flint PW, Francis HW, Haughey BH, et al, eds. Cummings Otolaryngology: Head and Neck Surgery. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 12.