Rheumatology
Sjögren Syndrome
쇼그렌증후군
Autoimmune exocrinopathy causing ocular and oral dryness
- How common
- 0.5–1% of population
- Typical age
- Ages 40–60 (9:1 female)
What is it?
Lymphocytic infiltration destroys salivary and lacrimal exocrine glands; some progress to systemic involvement with elevated lymphoma risk.
Commonly affected: Salivary/lacrimal glands → joints, lungs, kidneys, nerves
How it develops
- Glandular lymphocytic infiltrationCD4 T-cell and B-cell infiltration
- Apoptosis inductionGlandular cell death decreases secretion
- Autoantibody productionAnti-Ro (SS-A), anti-La (SS-B)
- Systemic & lymphoma riskJoint/lung/nerve involvement; 16x MALT lymphoma risk
Symptoms
- XerophthalmiaGritty sensation, redness
- XerostomiaDysphagia, taste changes, dental caries
- FatigueSevere fatigue and sleep disturbance
- Parotid swellingRecurrent bilateral parotid enlargement
- ArthralgiaNon-erosive polyarthralgia
How it is examined
- Schirmer testTear production <5mm in 5 min
- Oral exam + salivary flowUnstimulated saliva flow <0.1mL/min
- Autoantibodies + lip biopsyAnti-Ro/La, focus score ≥1
Imaging
Sialography or US assesses salivary architecture.
- Sialectasis
- Hypoechoic nodules
MRI sialography visualizes glands. PET if evaluating lymphoma.
- Glandular atrophy
- Cystic changes
Non-surgical care
- Artificial tears/oral lubricantsFrequent preservative-free tears
- Pilocarpine, cevimelineMuscarinic agonists to stimulate secretion
- HydroxychloroquineImproves arthralgia and fatigue
- Dental preventionFluoride and frequent dental visits
- Ophth (cyclosporine drops)Anti-inflammatory drops for severe dry eye
Conservative options are generally tried first. Medications listed here can have side effects — discuss them with your prescriber.
When surgery is considered
Severe systemic involvement or lymphoma
Procedures that may be discussed
- Rituximab (refractory)
- Methotrexate, azathioprine
- Oncology referral if lymphoma develops
Outlook
Generally good but 16x increased MALT lymphoma risk; needs regular monitoring.