Sjögren’s Syndrome

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)⁹ to 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 include¹⁰  autologous 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.