Symptoms of Sjögren's Syndrome
Sjögren's syndrome presents primarily with dry eyes and dry mouth, along with systemic manifestations including fatigue, joint pain, and swollen salivary glands, with women affected 20 times more frequently than men. 1
Ocular Symptoms
- Burning, itching, or gritty feeling in the eyes
- Foreign body sensation
- Light sensitivity (photophobia)
- Blurry vision
- Need for frequent use of eye drops 1
- Decreased tear production (measured by Schirmer test ≤5 mm/5 minutes) 1
- Ocular surface damage (detected through ocular staining) 1
Oral Symptoms
- Difficulty swallowing dry foods without liquids
- Frequent need to sip water
- Burning sensation in the mouth
- Dental problems (increased caries risk)
- Cracked, chipped teeth
- Gum inflammation
- Mouth sores
- Swollen salivary glands 1
- Decreased salivary flow (≤0.1 ml/minute) 1
Systemic Manifestations
- Persistent fatigue
- Joint and muscle pain (arthralgia, myalgia)
- Raynaud's phenomenon
- Numbness or tingling in extremities
- Dry skin
- Vaginal dryness 1
Extraglandular Manifestations
- Respiratory symptoms (may require pulmonary function tests)
- Renal involvement
- Gastrointestinal symptoms
- Hematological abnormalities
- Cardiovascular manifestations
- Reproductive system issues 1
Diagnostic Considerations
The American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) criteria require a score of ≥4 based on five weighted criteria:
- Anti-SSA/Ro antibody positivity (3 points)
- Focal lymphocytic sialadenitis on biopsy (3 points)
- Abnormal ocular staining score (1 point)
- Schirmer test ≤5 mm/5 minutes (1 point)
- Unstimulated salivary flow rate ≤0.1 ml/minute (1 point) 1
Important Clinical Pearls
- Approximately 10% of patients with clinically significant dry eye have underlying Sjögren's syndrome 2
- Many patients may not have been diagnosed at the time they present with dry eye complaints 2
- The prevalence is approximately 0.4% of the population with a female-to-male ratio of 20:1 1
- Primary Sjögren's syndrome may be associated with thyroid dysfunction or autoimmune thyroiditis 2
- Secondary Sjögren's syndrome occurs with other autoimmune diseases like rheumatoid arthritis, scleroderma, or systemic lupus erythematosus 2
- Patients are at increased risk for potentially life-threatening vasculitic or lymphoproliferative disorders 2
- Decreased C4 levels at diagnosis indicate higher risk of developing lymphoma 2
Red Flags Requiring Immediate Attention
- Worsening eye or mouth dryness
- New or worsening joint pain, rash, or fatigue
- Persistent dry cough or shortness of breath
- Unexplained fever or swollen glands
- Severe abdominal pain or yellowing of skin 1
Sjögren's syndrome should be suspected in nonelderly women with rapid onset or marked severity of intrinsic tear-production deficiency. Co-management with a rheumatologist is essential due to the many possible systemic complications 2, 1.