Sjögren’s Syndrome - Scope Connect

Introduction

Sjögren’s syndrome is a long-lasting autoimmune disease where the body’s immune system attacks its own glands that produce and control moisture. The glands most often affected are lacrimal in the eyes (makes tears) and salivary in the mouth (makes saliva). The glands that make tears are often affected, leading to problems like dry eyes, inflammation of the conjunctiva, and issues such as corneal ulcers and scleritis.¹

Sjögren’s syndrome comes in two types: primary and secondary. Primary Sjögren’s occurs on its own, while secondary Sjögren’s occurs with another autoimmune disease like rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE).² This damage is caused by certain types of white blood cells called CD4+ T cells and B cells.

The prevalence of Sjögren’s in the USA is second only to SLE in terms of common rheumatology disorders affecting 0.1-4% of the population, approximately 4 million people.³ It affects females much more than males (9:1) with a typical onset in the 4^th to 5^th decade of life. In the UK it affects approximately 0.6% of adults with 90% of patients being female.⁴

In Sjögren’s syndrome, the eyes are greatly impacted because there is less tear production and alterations in tear composition. When tear production decreases, vision may be affected and patients can complain of eye discomfort and vision.

Salivary and tear glands. Source stepwards.com

Clinical Evaluation

Risk Factors

Primary Sjögren’s may have no known trigger or cause, but a 2023 review found the following:⁵

Infection possibly leading to overstimulation of the immune system
Positive family history of autoimmune disease
History of pregnancy (weak association)
Genetic changes in IRF5 gene affecting the immune system
Negative stressful life events

The risk factors for secondary Sjögren’s are autoimmune diseases and some viral infections:⁶

Rheumatoid arthritis (RA) (most common)
Systemic lupus erythematosus (SLE)
Scleroderma
Hepatitis C
HIV

According to the American-European classification system (as modified by Tzioufas and Voulgarelis,⁷) diagnosis of primary Sjögren syndrome requires at least four of the criteria listed below; in addition, either criterion number 5 or criterion number 6 must be included. Sjögren syndrome can be diagnosed in patients who have no sicca symptoms if three of the four objective criteria are fulfilled. The criteria are as follows:

Ocular symptoms – Dry eyes for more than 3 months, foreign-body sensation, use of tear substitutes more than 3 times daily
Oral symptoms – Feeling of dry mouth, recurrently swollen salivary glands, frequent use of liquids to aid swallowing
Ocular signs – Schirmer test performed without anesthesia (< 5 mm in 5 min), positive vital dye staining results
Oral signs – Abnormal findings for salivary scintigraphy, parotid sialography, and sialometry (unstimulated salivary flow < 1.5 mL in 15 min)
Positive minor salivary gland biopsy findings
Positive anti–SSA or anti–SSB antibody results

Signs 

The ocular signs of Sjögren’s syndrome may include:⁸

Dry eyes (keratoconjunctivitis sicca)
Redness and irritation of the eyes (conjunctivitis)
Sensitivity to light (photophobia)
Grittiness or feeling of sand in the eyes
Blurred vision
Corneal ulcers or abrasions
Conjunctival scarring
Scleritis (inflammation of the sclera)
Corneal melting or perforation (in severe cases)
Reduced tear production (measured by Schirmer’s test)
Abnormalities in tear film quality or stability (measured by tear breakup time)
Conjunctival staining with vital dyes (e.g., fluorescein or lissamine green)

These signs may vary in severity and can impact visual function and ocular comfort in individuals with Sjögren’s syndrome.

Systemic lupus erythematous. Source www.treatSLE.com

Symptoms

The symptoms of Sjögren’s syndrome may include:²

Dry mouth (xerostomia)
Dry eyes (keratoconjunctivitis sicca)
Eye irritation, redness, or burning sensation
Difficulty swallowing or speaking due to dryness
Dry or cracked lips
Sore throat
Dental decay or cavities
Fatigue
Joint pain or swelling (arthralgia or arthritis)
Muscle pain or weakness
Skin rashes or dry skin
Vaginal dryness or discomfort
Persistent cough
Difficulty concentrating or memory problems
Swollen salivary glands, particularly around the jaw or neck areaThe symptoms of Sjögren’s syndrome may include:²

These symptoms can vary in severity and may come and resolve over time.

Source About.com Health

Investigations

Eyecare clinicians suspecting that Sjogren’s syndrome is causing their patient’s ocular problems may consider the following:

Detailed history

This is important as there may be an associated systemic problem.

This could include careful questioning around whether the patient has a dry eye and dry mouth and systemic aches and pains. At this stage general observation of the patient to look for signs of facial skin rashes (related to SLE) or joint deformations (RA).

Examination

Objective testing for ocular surface disease in Sjögren’s syndrome includes tear film evaluation using techniques such as tear breakup time (TBUT), Schirmer’s test, ocular surface staining with vital dyes (e.g., fluorescein and lissamine green), and assessment of meibomian gland function. Additional testing may include measurement of tear osmolarity, tear inflammatory cytokines, and imaging modalities such as optical coherence tomography (OCT) to evaluate corneal thickness and epithelial integrity.

Slit Lamp Examination

This would be the clinicians normal work up (least invasive first) for dry eye patients to include assessment of

Tear film quantity (non-invasive but may also include Schirmer’s strips)
Tear film quality (non-invasive)
Lids and lashes
Conjunctiva
Cornea
Fluorescein/lissamine as appropriate

Management & Advice

Due to the fact that these patients are likely to have an underlying systemic condition, it is probably more appropriate that they are managed and advised by a healthcare team, typically involving the optometrist, the ophthalmologist and a rheumatologist.
Suggested management strategies and advice for patients with suspected Sjögren’s syndrome may include:

1. Confirm the Diagnosis
Obtain a definitive diagnosis through a combination of medical history, physical examination, blood tests (e.g., anti-SSA/Ro, anti-SSB/La antibodies), and possibly a lip biopsy or other specialised tests.

2. Symptom Management (Ocular)
Dry Eyes: Use artificial tears, preferable preservative free, humidifiers, and avoid dry environments. Prescription medications such as cyclosporine eye drops may be prescribed for severe dry eye symptoms.

3. Eye Care: Regular eye exams by an optometrist/ophthalmologist should be considered to monitor eye health, managing the dry eye symptoms, and preventing complications such as corneal ulcers or infections.

4. Dental Care
Regular dental visits are important to prevent dental decay and infections due to reduced saliva production.

5. Systemic Complications Monitoring
Monitor for systemic complications

6. Patient Education
Provide information about Sjögren’s syndrome and encourage patients to be proactive in managing their symptoms and to communicate openly with their healthcare team.

7. Supportive Care
Offer support groups or counselling services to help patients cope with the impact of living with a chronic autoimmune condition.

8. Regular Follow-up
Schedule regular follow-up appointments to assess disease activity, adjust treatment as needed, and address any new or worsening symptoms.

Overall, the management of Sjögren’s syndrome focuses on symptom relief, preventing complications, and improving quality of life for patients. It is important for healthcare providers to work collaboratively with patients to tailor treatment plans to their individual needs and preferences.

Non-Pharmacological Management/Treatment

Management of ocular manifestations in Sjögren’s syndrome aims to alleviate symptoms, improve ocular surface health, and prevent complications. The mainstay of management for the ocular effects of Sjögren’s Syndrome is the use of ocular lubricants. These often need to be used frequently and a preservative free option should be considered by the clinician as recommended by the Tear Film and Ocular Surface Society (TFOS)⁹to minimise ocular irritation and toxicity. This is particularly important as the ocular effects of Sjögren’s syndrome may lead to ongoing problems with comfort and vision. Chronic conjunctivitis may lead to conjunctival scarring and fibrosis, further exacerbating dry eye symptoms and impairing ocular surface health.

Corneal involvement is a significant concern in Sjögren’s syndrome, as chronic dryness and inflammation predispose patients to corneal complications such as epithelial defects, erosions, ulcers, and perforations. Without proper ocular lubrication and protection, the cornea may become vulnerable to mechanical trauma, microbial colonization, and delayed epithelial healing. Corneal ulcers, if left untreated, can progress to sight-threatening complications such as corneal melting and perforation.

Pharmacological Treatments

Treatment strategies often involve a combination of lubricating eye drops, anti-inflammatory medications, and systemic immunosuppressive therapy.

In addition to lubricating eye drops, anti-inflammatory medications such as corticosteroids and nonsteroidal anti-inflammatory drugs (NSAIDs) may be used to control ocular inflammation and reduce symptoms. Topical corticosteroids are effective in managing acute exacerbations of ocular inflammation, but long-term use should be limited due to the risk of steroid-induced complications such as elevated intraocular pressure and cataract formation. NSAIDs, on the other hand, inhibit prostaglandin production and offer symptomatic relief of ocular pain and inflammation without the systemic side effects associated with corticosteroids, but clinicians should consider the possibility of exacerbating corneal problems with these family of medications. Other topical treatments may include¹⁰autologous serums, allogenic serums, cyclosporin eye drops, lifitegrast eye drops and diquafosol sodium eyedrops (approved in Japan). Other interventional treatments may include the use of punctal plugs to preserve patient’s tears, and amniotic membranes to promote wound healing growth factors and reduce pro-inflammatory cytokines.

Systemic immunosuppressive therapy may be indicated for patients with severe ocular manifestations of Sjögren’s syndrome refractory to topical treatments. Immunosuppressive agents such as hydroxychloroquine, methotrexate, cyclosporine, and rituximab have been used to reduce systemic inflammation and preserve ocular surface function in patients with Sjögren’s syndrome.

This article serves as an overview of the condition and treatment options. It does not serve as a clinical guidance. Eyecare provider guidelines should be used when managing patients.