Sjögren's Syndrome

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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 Quick Facts

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

Sjögren's Symptoms - Desert Within

Key Manifestations:

Feature TypeCommon Symptoms
Glandular (Sicca Symptoms)* Xerostomia (dry mouth): difficulty swallowing, dental caries, oral candidiasis.
* Keratoconjunctivitis Sicca (KCS): dry eyes, gritty sensation, redness, corneal ulcers.
* Salivary/Parotid gland enlargement: often bilateral, recurrent.
Extraglandular* Articular: arthralgia, non-erosive arthritis.
* Pulmonary: interstitial lung disease.
* Renal: interstitial nephritis, renal tubular acidosis.
* Neurological: peripheral neuropathy.
* Cutaneous: purpura, Raynaud's phenomenon.
* Vascular: vasculitis.
* Thyroid: autoimmune thyroiditis.

⭐ 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)
      • Sjögren's syndrome salivary gland biopsy
    • 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.

Practice Questions: Sjögren's Syndrome

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A 45-year-old female presents with dry eyes, dry mouth, and joint pain. Which of the following antibodies is most indicative of the suspected condition?

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Flashcards: Sjögren's Syndrome

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The _____ is progressively pushed upwards and backward threatening the airway, in Ludwig's angina

TAP TO REVEAL ANSWER

The _____ is progressively pushed upwards and backward threatening the airway, in Ludwig's angina

tongue

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