Autoimmune Diseases Causing Dry Eye and Dry Mouth (Sicca) Symptoms
Sjögren's syndrome is the primary autoimmune disease causing both dry eye and dry mouth symptoms, but several other systemic autoimmune diseases also commonly present with sicca manifestations. 1, 2
Primary Autoimmune Diseases Associated with Sicca Symptoms
Sjögren's Syndrome
- Sjögren's syndrome is defined specifically by the combination of dry eye (keratoconjunctivitis sicca) and dry mouth (xerostomia) associated with systemic immune dysfunction. 1
- Affects approximately 0.4% of the population, with an annual incidence of 5.3 per 100,000 and prevalence of 92.8 cases per 100,000. 1
- Has a striking female predominance with a female-to-male ratio of 20:1. 1
- Can occur as primary Sjögren's syndrome (in previously healthy individuals) or secondary Sjögren's syndrome (associated with other autoimmune diseases). 1, 2
Secondary Sjögren's Syndrome - Associated Autoimmune Diseases
When Sjögren's syndrome occurs alongside other systemic autoimmune disorders, the following conditions are most commonly implicated: 2
- Rheumatoid arthritis 2, 3
- Systemic lupus erythematosus (SLE) 2, 3
- Progressive systemic sclerosis (scleroderma) 2, 3
- Dermatomyositis 2
Other Autoimmune Diseases with Dry Eye Manifestations
Multiple systemic autoimmune diseases beyond Sjögren's syndrome are associated with moderate to severe dry eye disease: 4, 5
- Rheumatoid arthritis - frequently presents with dry eye even without concurrent Sjögren's syndrome 3, 4
- Systemic lupus erythematosus - significant overlap in clinical manifestations with Sjögren's syndrome 2, 3
- Systemic sclerosis - associated with chronic dry eye disease 3, 4
Clinical Recognition and Diagnostic Considerations
High-Risk Patient Profile
Suspect an underlying systemic autoimmune disease in patients presenting with: 4
- Female gender, age 30-60 years 4
- Family history of autoimmunity 4
- Dry eye symptoms plus extraocular manifestations including arthralgias, dry mouth, unexplained weight loss, hair loss, chronic fatigue, heat or cold intolerance, insomnia, and mood disorders 4
Pathophysiology
- Characterized by focal lymphocytic infiltration of lacrimal and salivary glands with secondary compromise of gland function 1
- Systemic immune dysregulation in autoimmune diseases can amplify inflammatory stress responses on the ocular surface, leading to chronic dry eye disease 3
- Dysfunctional, hyper-responsive immune cells recruited to the ocular surface can disrupt tear stability 3
Important Clinical Pitfalls
Serious Complications Requiring Vigilance
Patients with autoimmune-related dry eye are at risk for: 1, 2
- Ocular complications: scleritis, sterile keratitis, uveitis, corneal ulceration, and corneal melting 1, 4
- Systemic complications: potentially life-threatening vasculitic or lymphoproliferative disorders 1
- Lymphoma risk: 2-5% of Sjögren's patients develop lymphoma; patients with decreased C4 levels at diagnosis have higher lymphoma risk 1
- Rapid tooth loss and oral candidiasis from oral dryness 2
When to Refer
Patients with dry eye should be evaluated by an ophthalmologist for: 1
- Moderate or severe pain
- Lack of response to therapy
- Corneal infiltration or ulceration
- Progressive conjunctival scarring
- Vision loss
All Sjögren's syndrome patients should be co-managed with a rheumatologist due to multiple possible comorbid systemic conditions and the need for systemic monitoring. 1