U01.11.040 Sjögren syndrome

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Learning Objectives

Explain the autoimmune destruction of exocrine glands and its clinical consequences. Identify the specific serologic markers (Anti-Ro/SSA and Anti-La/SSB) and diagnostic criteria, including salivary gland biopsy. Recognize the serious complications, such as MALT lymphoma and neonatal lupus risks.

1. Pathophysiology & Epidemiology

Sjögren syndrome is a chronic autoimmune disorder characterized by lymphocytic infiltration (predominantly T cells) and destruction of the exocrine glands, specifically the lacrimal and salivary glands. It predominantly affects females between 40 and 60 years old.

  • Primary vs. Secondary: It can occur as an isolated condition (1°) or secondary to other autoimmune diseases (2°), most commonly Rheumatoid Arthritis, SLE, or Systemic Sclerosis.
  • Diagnosis: Confirmed by focal lymphocytic sialadenitis on labial salivary gland biopsy.

2. Clinical Findings

The “sicca” symptoms (dryness) are the hallmark of the disease, leading to significant mucosal and ocular damage.

System Clinical Finding High-Yield Description
Eyes Keratoconjunctivitis sicca Decreased tears $Latex\rightarrow$ “gritty” or “sandy” feeling; corneal damage.
Mouth Xerostomia Decreased saliva $Latex\rightarrow$ fissuring of the tongue and mucosal atrophy.
Glands Parotid Enlargement Typically bilateral and painless.
Other Dyspareunia & Joints Decreased vaginal lubrication and inflammatory joint pain.

3. Serology & Complications

Sjögren syndrome is associated with specific autoantibodies that are critical for board exams.

  • SS-A (anti-Ro) & SS-B (anti-La): Antibodies against ribonucleoprotein.
    • Note: These can also be found in SLE.
    • Pregnancy Risk: Anti-Ro/SSA can cross the placenta, leading to Neonatal Lupus (classic finding: congenital heart block).
  • MALT Lymphoma: Chronic stimulation of B cells in the parotid glands leads to a significantly increased risk of B-cell (MALT) lymphoma.
  • Dental Caries: Lack of saliva’s protective/antibacterial properties leads to rapid tooth decay.

Activity: Autoantibody Matching Game

High-Yield Mnemonics:

  • Sjögren’s Signs: Can’t See (Dry eyes), Can’t Spit (Dry mouth), Can’t S… (Dyspareunia/Dryness elsewhere).
  • Anti-Ro/La: “Ro” is for the baby (Neonatal Lupus/Heart Block); “La” is the accompaniment.

Activity: