What is the workup for Sjogren (Sjögren's) syndrome?

Diagnostic Workup for Sjögren's Syndrome

The comprehensive workup for Sjögren's syndrome should include serologic testing for autoantibodies, objective assessment of lacrimal and salivary gland function, and minor salivary gland biopsy to confirm diagnosis. 1

Initial Evaluation

• Detailed history focusing on key symptoms including oral dryness (xerostomia), ocular dryness (keratoconjunctivitis sicca), fatigue, and musculoskeletal pain 1, 2
• Baseline chest radiography should be performed in all patients with suspected or confirmed Sjögren's syndrome to screen for pulmonary involvement 3
• Complete pulmonary function tests (PFTs) should be considered even in asymptomatic patients to establish baseline measurements 3

Serologic Testing

• Test for traditional autoantibodies:
  • Anti-SSA/Ro and anti-SSB/La antibodies
  • Antinuclear antibody (ANA)
  • Rheumatoid factor (RF) 1, 4
• Consider testing for novel biomarkers that may appear earlier in disease course:
  • Antibodies to SP-1 (salivary gland protein-1)
  • PSP (parotid secretory protein)
  • CA-6 (carbonic anhydrase VI) 5

Objective Assessment of Glandular Function

Ocular Assessment

• Tear break-up time test
• Ocular surface dye staining
• Schirmer test (measures tear production)
• Tear osmolarity test 3, 1

Oral/Salivary Assessment

• Baseline evaluation of salivary gland function by measuring whole salivary flows 6
• Salivary scintigraphy may be considered to assess gland function 6

Histopathologic Examination

• Minor salivary gland biopsy is the gold standard for diagnosis, showing focal lymphocytic infiltrates 2, 4
• A focus score (number of lymphocytic foci per 4 mm² of glandular tissue) should be calculated, with ≥1 focus considered positive 4

Systemic Evaluation

• Monitor for signs of systemic involvement:
  • Unexplained weight loss
  • Fevers
  • Night sweats
  • Lymphadenopathy
  • Parotid gland enlargement 1
• For patients with suspected pulmonary involvement, high-resolution CT (HRCT) is recommended over plain chest X-ray 1
• Consider PET scan for patients with pulmonary lesions 1
• Biopsy should be performed for patients with lymphadenopathy, growing lung nodules, or progressive cystic lung disease 1

Disease Activity Assessment

• Use validated tools such as EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) to measure systemic activity 6, 3
• Evaluate severity of dryness, fatigue, and pain using EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) 6

Exclusion of Mimics

• Rule out conditions that can mimic Sjögren's syndrome:
  • Infections with HIV, human T-lymphotropic virus type I, or hepatitis C virus 2
  • Medication-induced sicca symptoms
  • Age-related dryness 4
  • Fibromyalgia (which can present with similar symptoms) 2

Monitoring for Complications

• Regular assessment for lymphoma development (2-5% risk) through clinical examination for lymphadenopathy and monitoring of laboratory parameters 6
• For patients with pulmonary involvement, repeat PFTs every 6-12 months 3
• Regular ophthalmologic follow-up to monitor for corneal complications 1

The diagnostic approach should be systematic and comprehensive, as early diagnosis allows for timely intervention to manage symptoms and potentially slow disease progression 5.