What is the initial workup for a patient suspected of having Sjogren's (Sjogren's syndrome)?

Initial Workup for Suspected Sjögren's Syndrome

The initial workup for a patient suspected of having Sjögren's syndrome should include serological testing for anti-SSA/Ro and anti-SSB/La antibodies, antinuclear antibody (ANA), rheumatoid factor (RF), objective assessment of ocular and oral dryness, and a labial salivary gland biopsy for histological confirmation. 1

Diagnostic Approach

Step 1: Symptom Assessment

• Evaluate for key symptoms:
  • Oral symptoms: Dry mouth, need for liquids to swallow food, frequent water sipping, burning sensation, angular cheilitis, frequent dental cavities, tooth erosion, gum inflammation 2
  • Ocular symptoms: Dry eyes, foreign body sensation, light sensitivity, frequent use of eye drops, blurry vision 2
  • Other symptoms: Parotid/submandibular gland swelling, vaginal dryness, skin dryness, peripheral neuropathy, fatigue, joint/muscle pain, Raynaud's phenomenon 2

Step 2: Essential Laboratory Tests

• Autoantibody testing:
  • Anti-SSA/Ro antibodies (weighted 3 points in diagnostic criteria)
  • Anti-SSB/La antibodies
  • Antinuclear antibody (ANA)
  • Rheumatoid factor (RF) 1
• Additional blood tests:
  • Complete blood count
  • Comprehensive metabolic panel
  • Erythrocyte sedimentation rate (ESR)
  • C-reactive protein (CRP) 1

Step 3: Objective Assessment of Glandular Function

• Ocular tests:
  • Schirmer test (≤5 mm/5 minutes = 1 point in diagnostic criteria)
  • Ocular staining score (abnormal = 1 point in diagnostic criteria) 1
• Oral tests:
  • Unstimulated salivary flow rate (≤0.1 ml/minute = 1 point in diagnostic criteria) 1
  • Minor salivary gland biopsy (focal lymphocytic sialadenitis = 3 points in diagnostic criteria) 1, 3
• Additional imaging:
  • Major salivary gland ultrasound (growing importance as diagnostic tool) 3

Step 4: Exclusion of Other Conditions

• Rule out:
  • Pre-existing lymphoma
  • Hepatitis C infection
  • HIV infection 4
  • Other causes of sicca symptoms (medications, radiation therapy)

Diagnostic Criteria

According to the American College of Rheumatology and European League Against Rheumatism criteria, a score of ≥4 based on the following weighted items is required for diagnosis 1:

Criterion	Weight
Anti-SSA/Ro antibody positivity	3
Focal lymphocytic sialadenitis	3
Abnormal ocular staining score	1
Schirmer test ≤5 mm/5 minutes	1
Unstimulated salivary flow rate ≤0.1 ml/minute	1

Additional Evaluation for Systemic Involvement

• Pulmonary assessment:

  • Baseline pulmonary function tests (PFTs) including spirometry and DLCO
  • Chest radiography 1
  • High-resolution CT (HRCT) for patients with respiratory symptoms or abnormal PFTs 1

• Other system evaluations based on symptoms:

  • Neurological examination for peripheral neuropathy
  • Joint examination for arthritis
  • Skin examination for vasculitis 2, 4

Important Considerations

• Early diagnosis is crucial: Sjögren's syndrome may initially present with non-sicca (systemic) manifestations before the onset of obvious dryness symptoms ("occult" Sjögren's) 5

• Lymphoma risk: Patients have a markedly increased risk of lymphoma. Enlarged lymph nodes or persistently enlarged salivary glands require further evaluation 4

• Multidisciplinary approach: Close collaboration between rheumatology, ophthalmology, dentistry, and other specialties is essential for comprehensive evaluation 1, 5

• Primary vs. Secondary Sjögren's: Determine if Sjögren's exists as a primary condition or in association with other autoimmune disorders (secondary Sjögren's) such as rheumatoid arthritis, SLE, scleroderma, or dermatomyositis 4