Sjögren's Syndrome

Sjögren's Syndrome - Dry Spell Defined

• Sjögren's Syndrome (SS): A chronic, slowly progressive, systemic autoimmune disease.
• Primarily targets exocrine glands, leading to sicca symptoms:
  • Xerostomia (dry mouth) due to salivary gland dysfunction.
  • Keratoconjunctivitis sicca (KCS) / dry eyes due to lacrimal gland dysfunction.
• Epidemiology:
  • Predominantly affects women (F:M ratio approx. 9:1).
  • Peak age of onset: 40-60 years.
  • Primary SS: occurs in isolation.
  • Secondary SS: coexists with other autoimmune diseases (e.g., Rheumatoid Arthritis, SLE, Scleroderma).
• Core Pathomechanism:

  ⭐ Characterized by lymphocytic infiltration (mainly CD4+ T cells and B cells) of exocrine glands, primarily salivary and lacrimal, leading to glandular dysfunction and destruction.

• Etiology: Complex interplay of genetic (e.g., HLA-DRB1, HLA-DQ) and environmental factors.

Sjögren's Syndrome - Sicca Saga & Beyond

• Autoimmune exocrinopathy: lymphocytic infiltration of lacrimal & salivary glands.
• Primary Sjögren's: occurs alone.
• Secondary Sjögren's: with other autoimmune diseases (RA, SLE, Scleroderma).

Glandular Manifestations (Sicca Complex):

• Xerophthalmia (Dry Eyes):
  • Gritty sensation, redness, photosensitivity.
  • Schirmer's test: < 5 mm in 5 min.
  • Rose Bengal / Lissamine Green staining.
• Xerostomia (Dry Mouth):
  • Difficulty swallowing, dental caries, oral candidiasis.
  • Salivary gland swelling (parotid).
  • ↓ unstimulated salivary flow.

Extraglandular Manifestations:

• Musculoskeletal: Arthralgia, non-erosive arthritis.
• Cutaneous: Raynaud's phenomenon, purpura, vasculitis.
• Pulmonary: Interstitial lung disease (ILD), xerotrachea.
• Renal: Interstitial nephritis, renal tubular acidosis.
• Neurological: Peripheral neuropathy, CNS involvement (rare).
• Hematological: Anemia, leukopenia, ↑ risk of lymphoma (MALT).

⭐ Besides sicca symptoms, common extraglandular manifestations include arthritis, Raynaud's phenomenon, and pulmonary involvement.

Antibodies:

• Anti-Ro (SSA): ~70%
• Anti-La (SSB): ~40%
• ANA, RF often positive.

📌 Mnemonic: Sicca, Salivary gland swelling, SSA/SSB antibodies, Secondary to other diseases (sometimes).

Sjögren's Syndrome - Cracking the Case

For patients with ≥1 sicca symptom. Diagnosis hinges on ACR/EULAR 2016 criteria (score ≥4 needed).

• Key Diagnostic Components (Points):
  • Ocular Signs (1 pt each if present in ≥1 eye):
    • Schirmer’s test: ≤5 mm in 5 min
    • Ocular Staining Score (OSS): ≥5 (or van Bijsterveld score ≥4)
  • Oral Signs:
    • Unstimulated whole salivary flow: ≤0.1 mL/min (1 pt)
    • Labial Salivary Gland (LSG) Biopsy: Focal lymphocytic sialadenitis, focus score ≥1 focus/4mm² (3 pts)
  • Autoantibodies:
    • Anti-Ro/SSA positive (3 pts)

⭐ Positive anti-Ro/SSA and/or anti-La/SSB antibodies, or a positive labial salivary gland biopsy (focus score ≥1), are key for diagnosis under ACR/EULAR criteria.

Sjögren's Syndrome - Taming Thirst & Troubles

• Autoimmune; exocrine gland destruction (lacrimal, salivary).
• Symptoms: Dry eyes (keratoconjunctivitis sicca), dry mouth (xerostomia), fatigue, arthralgia.
• Diagnosis: Schirmer test (↓ tear production), salivary gland biopsy (focal lymphocytic sialadenitis), autoantibodies (Anti-Ro/SSA, Anti-La/SSB).
• Management:
  • Symptomatic: Artificial tears/saliva, pilocarpine, cevimeline.
  • Systemic: Hydroxychloroquine, corticosteroids, immunosuppressants (e.g., methotrexate, rituximab) for severe/systemic disease.

⭐ Patients have a significantly increased risk (up to 44-fold) of developing Non-Hodgkin Lymphoma, especially MALT type.

• Complications: Dental caries, corneal ulcers, Raynaud's, vasculitis, neuropathy, lymphoma. 📌 Sicca, Salivary, Serology (SSA/SSB).

High‑Yield Points - ⚡ Biggest Takeaways

• Autoimmune exocrinopathy targeting salivary & lacrimal glands.
• Presents with keratoconjunctivitis sicca (dry eyes) & xerostomia (dry mouth).
• Anti-Ro/SSA & Anti-La/SSB antibodies are highly specific.
• Significant ↑ risk of B-cell non-Hodgkin lymphoma (MALT type).
• Diagnosis aided by Schirmer's test & lip biopsy (focal lymphocytic sialadenitis).
• May be primary or secondary to other autoimmune diseases (e.g., RA, SLE).
• Extraglandular features include arthritis, Raynaud's, neuropathy.