What is the workup for Sjogren's syndrome?

Diagnostic Workup for Sjögren's Syndrome

The diagnostic workup for Sjögren's syndrome should include serological testing for anti-SSA/Ro and anti-SSB/La antibodies, ANA, and RF, along with objective assessment of lacrimal and salivary gland function, and minor salivary gland biopsy. 1

Initial Evaluation

• History and Symptom Assessment

  • Evaluate for key symptoms including dry mouth (need for liquids to swallow food, frequent water sipping, burning sensation, dental cavities) and dry eyes (sensation of dryness, irritation, foreign body sensation, light sensitivity) 2
  • Assess for systemic manifestations such as joint/muscle pain, skin rashes, chronic dry cough, vaginal dryness, extremity numbness/tingling, and fatigue 3

• Serological Testing

  • Test for traditional biomarkers: anti-SSA/Ro, anti-SSB/La, ANA, and RF 1, 2
  • Consider newer biomarkers: antibodies to SP-1 (salivary gland protein-1), PSP (parotid secretory protein), and CA-6 (carbonic anhydrase VI), which may appear earlier in disease course 3

Objective Assessments

• Ocular Evaluation

  • Tear break-up time test 1
  • Ocular surface dye staining 4
  • Schirmer test to measure tear production 4, 5
  • Tear osmolarity test 4

• Oral/Salivary Evaluation

  • Sialometry (measurement of salivary flow) 5
  • Minor salivary gland biopsy - considered the gold standard for diagnosis 5, 6
    • Assess for focal lymphocytic infiltrates with focus score calculation 6
  • Major salivary gland ultrasound - increasingly important diagnostic tool 5

Systemic Evaluation

• Pulmonary Assessment

  • Baseline chest radiography for all patients with suspected or confirmed Sjögren's syndrome 4
  • Complete pulmonary function tests (PFTs), even in asymptomatic patients 4
  • High-resolution CT (HRCT) for patients with respiratory symptoms 4

• Screening for Lymphoproliferative Complications

  • Monitor for unexplained weight loss, fevers, night sweats, lymphadenopathy, and/or parotitis 7
  • Consider HRCT chest scan rather than plain chest X-ray 7
  • Consider PET scan for patients with pulmonary lesions (nodules >8mm, consolidations, or lymphadenopathy) 7
  • Biopsy for patients with lymphadenopathy, growing lung nodules, and/or progressive cystic lung disease 7

Diagnostic Criteria

• The 2016 ACR/EULAR classification criteria use a weighted scoring system:
  • Anti-SSA/Ro antibody positivity scores 3 points 2
  • Focal lymphocytic sialadenitis with focus score ≥1 foci/4 mm² scores 3 points 2
  • Abnormal ocular staining score ≥5 scores 1 point 1
  • Schirmer test ≤5 mm/5 minutes scores 1 point 1
  • Unstimulated salivary flow rate ≤0.1 ml/minute scores 1 point 1
  • A total score of ≥4 meets criteria for primary Sjögren's syndrome 2

Important Considerations

• Exclude other conditions that can cause sicca symptoms:

  • Infections with HIV, HTLV-1, or hepatitis C virus 8
  • Medication side effects 6
  • Age-related dryness 6

• Patients with fibromyalgia-like symptoms (arthralgia, myalgia, fatigue) with oral and ocular dryness and circulating ANAs present diagnostic challenges 8

• A multidisciplinary approach involving rheumatologists, ophthalmologists, and dental professionals is essential for both diagnosis and ongoing management 2, 9