Sjögren's Syndrome

Sjögren's Intro - Dry Spells Ahead

• Definition: A chronic, systemic autoimmune disease primarily affecting exocrine glands, especially lacrimal (tear) and salivary (saliva) glands, leading to dryness (sicca symptoms).
• Types:
  • Primary Sjögren's: Occurs in the absence of other autoimmune connective tissue diseases.
  • Secondary Sjögren's: Occurs in conjunction with another autoimmune disease (e.g., Rheumatoid Arthritis, SLE, Systemic Sclerosis).
• Pathogenesis: Key mechanisms include:
  • Lymphocytic infiltration (predominantly CD4+ T cells and B cells) of exocrine glands.
  • B-cell hyperactivity and production of autoantibodies, notably anti-Ro/SSA and anti-La/SSB.
• Genetic Predisposition: Strong association with specific HLA alleles, including HLA-DR2, HLA-DR3, and HLA-B8.

⭐ Sjögren's Syndrome is the second most common autoimmune rheumatic disease after Rheumatoid Arthritis (RA).

Sjögren's Symptoms - Desert Within

Key Manifestations:

⭐ The most feared complication of Sjögren's Syndrome is the significantly increased risk (approximately 5-10% or 15-20 times higher than general population) of developing B-cell non-Hodgkin lymphoma, particularly MALT lymphoma (Mucosa-Associated Lymphoid Tissue lymphoma).

Sjögren's Diagnosis - Spotting Signs

• ACR/EULAR 2016 Criteria: Score ≥4 needed. Items (points):

  • Anti-SSA/Ro Ab (+): 3 pts
  • Labial Salivary Gland Biopsy (LSGB): Focal lymphocytic sialadenitis, focus score ≥1/4mm² (3 pts)
    • 
  • Ocular Staining Score (OSS) ≥5 (or van Bijsterveld ≥4): 1 pt
  • Schirmer's Test ≤5 mm/5 min: 1 pt
  • Unstimulated Salivary Flow ≤0.1 mL/min: 1 pt
  • 📌 ROSE-S (Criteria): Ro, Ocular, Salivary biopsy, Eye-Schirmer's, Salivary flow

• Diagnostic Flowchart (ACR/EULAR 2016 Simplified):

• Key Tests:
  • Serology: ANA, RF, Anti-Ro/SSA (+ specific), Anti-La/SSB.
  • Ocular: Schirmer's, Rose Bengal/Lissamine Green, TBUT.
  • Oral: Salivary flow, Sialography, Scintigraphy. LSGB (gold standard for salivary).

⭐ Positive anti-Ro/SSA in pregnancy: risk of neonatal lupus & congenital heart block in fetus.

Sjögren's Management - Moisture Mission

• Goals: Symptomatic relief, prevent complications, treat systemic issues.
• Sicca Symptoms:
  • Ocular: Artificial tears, ointments, topical cyclosporine, punctal occlusion.
  • Oral: Saliva substitutes, lozenges, oral hygiene. Sialogogues: Pilocarpine (5mg PO TID-QID), Cevimeline (30mg PO TID).
• Extraglandular Issues:
  • NSAIDs (arthralgia).
  • Hydroxychloroquine (fatigue, arthralgia, myalgia).
  • Corticosteroids (severe systemic).
  • Immunosuppressants (methotrexate, etc.) for organ-threatening disease.
• Biologics: Rituximab, Belimumab for severe, refractory disease/lymphoma.
• Monitoring: Regular follow-up (lymphoma, dental caries, corneal damage).

⭐ Hydroxychloroquine is often used as a first-line systemic agent for managing fatigue and arthralgia in Sjögren's Syndrome, but requires regular ophthalmic screening.

High-Yield Points - ⚡ Biggest Takeaways

• Autoimmune exocrinopathy targeting salivary & lacrimal glands, causing xerostomia & keratoconjunctivitis sicca.
• Characteristic autoantibodies include Anti-Ro (SSA) & Anti-La (SSB); often ANA positive.
• Schirmer's test (<5mm/5min) & Rose Bengal staining assess ocular dryness & damage.
• Minor salivary gland (lip) biopsy showing focal lymphocytic sialadenitis (focus score ≥1) is diagnostic.
• Significantly ↑ risk of B-cell non-Hodgkin lymphoma, especially MALT lymphoma of parotid.
• Occurs as Primary Sjögren's or Secondary to diseases like RA, SLE.